mcq of hematology

7/30/2019 Mcq of Hematology

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ABDULRAHMAN BASHIRE CHILDREN HOSPITAL -- BENGHAZI

1. The serum iron is often raised in the following condition:-

A. Anaemia of chronic disorders.B. Beta thalassemia.

C. Iron deficiency anaemia.D. Sideroblastic anaemia.

E. Congenital spherocytosis

Ans: -BD

2. All of the following statements are true Except: -A. Approximately 10% of RBCs are normally removed each day and replaced by the marrow to

maintain the RBC count

B. When the capacity of the heme-binding proteins in the plasma is exceeded, free hemoglobin

appears in the plasma

C. The most common cause of aplastic crisis is parvovirus B19 infection

D. The marrow can increase its output of RBCs two- to threefold acutelyE. The usual marrow response to a chronic hemolytic anemia is reflected by a reticulocyte index of

3-4Ans:- A

Normal RBC survival time is 110120 days (half-life, 5560 days), and approximately 1% of RBCs (thesenescent ones) are removed each day and replaced by the marrow to maintain the RBC count.

3. All of the following statements are true Except:

A. The anatomic site of hematopoiesis changes during gestation and the population of cells

generated at those sites are distinct

B. Few neutrophils are found in the fetal circulation until the third trimester

C. Thrombopoietin is the physiologic regulator of platelet production but does not act as a potentstimulator of all stages of megakaryocyte growth and development

D. Erythropoiesis in utero is controlled by erythroid growth factors produced solely by the fetus

E. Some HbA can be detected in even the smallest embryos

Ans:- C

Thrombopoietin (TPO) is the physiologic regulator of platelet production and acts as a potent stimulator of

all stages of megakaryocyte growth and development.

4. Regarding platelets:

A. They have a life span of approximately 10 daysB. Platelet production is controlled by specific cytokines

C. Platelets should be stored between 2 and 6 degrees CD. Platelets can be stored for a maximum of 48 hours

E. A pool of platelets transfused can produce an increment of 50 000 / ml if platelet consumption is

not an issue

Ans:-AB

Platelets are produced from megakaryocytes, which are regulated by thrombopoietin, a specific cytokine.

They have a lifespan of 10-12 days thereafter being destroyed in the spleen.

Platelet concentrates should be stored at around 20 degrees C and the pH kept between 6.2 and 7.8 - these

conditions reduce the risk of a change in morphology of the platelets. They should also be continuously

agitated to encourage gas exchange.

One pool of platelets gives on average, an increment of 10 000 / ml.

5. All of the following statements regarding iron deficiency are true Except::-A. Because absorption of dietary iron is assumed to be about 10%, a diet containing 80-100 mg of

iron daily is necessary for optimal nutrition


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B. Intense exercise conditioning may result in iron depletion in adolescent girls

C. Iron deficiency may have effects on neurologic and intellectual function

D. The level of serum ferritin provides a relatively accurate estimate of body iron stores in the

absence of inflammatory disease

E. The red cell distribution width (RDW) is elevated in iron deficiency but not in and

thalassemia trait

Ans:-A

6. The following is true of iron deficiency anaemia:-

A. Is the commonest cause of microcytic hypochromic anaemia in infancyB. Serum transferrin is the most reliable indicator other than BM aspiration, of total iron stores.

C. Is commonly present from 2 months of age upward in term infant.D. The ratio of MCV to the red blood cell count is useful in differentiation from thalassemia.

E. Decreased T cell function and cell mediated immunity is reported.

Ans:ADE

Low birthweight and unusual perinatal hemorrhage are associated with decreases in neonatal hemoglobin

mass and stores of iron. As the high hemoglobin concentration of the newborn infant falls during the first 2

3 mo of life, considerable iron is reclaimed and stored. These reclaimed stores usually are sufficient forblood formation in the first 69 mo of life in term infants. In low-birthweight infants or those with perinatal

blood loss, stored iron may be depleted earlier, and dietary sources become of paramount importance.

7. A 2 year old Pakistani boy has a haemoglobin of 8g/dl and an MCV of 65. The following tests are

essential:

A. Serum ferritinB. Serum B12

C. Serum folateD. Faecal occult blood

E. Haemoglobin electrophoresis

Ans:-ADEComments:In progressive iron deficiency, a sequence of biochemical and haematologic events occurs. First, the tissue

iron stores represented by bone marrow haemosiderin disappear. The level ofserum ferritin, an iron-storage

protein, provides a relatively accurate estimate of body iron stores in the absence of inflammatory disease.

Normal ranges are age dependent, and decreased levels accompany iron deficiency. Next, there is a decrease

in serum iron (also age dependent), the iron-binding capacity of the serum (serum transferrin) increases, and

the percent saturation (transferrin saturation) falls below normal (also varies with age). When the availability

of iron becomes rate limiting for haemoglobin synthesis, a moderate accumulation of heme precursors, free

erythrocyte protoporphyrins (FEP), results. As the deficiency progresses, the red blood cells (RBCs) becomesmaller than normal and their haemoglobin content decreases. The morphologic characteristics of RBCs are

best quantified by the determination of mean corpuscular haemoglobin (MCH) and mean corpuscularvolume (MCV). Developmental changes in MCV require the use of age-related standards for diagnosis of

microcytosis . With increasing deficiency the RBCs become deformed and misshapen and presentcharacteristic microcytosis, hypochromia, poikilocytosis and increased red cell distribution width (RDW).

Iron deficiency is much commoner in more economically deprived communities, with up to 50% being

affected in some inner city areas.Detailed investigation is therefore unnecessary in this child, unless he fails

to respond to a trial of iron therapy.

8. Iron deficiency anaemia;-A. Is characterized by low serum iron and low TIBC

B. Is associated with pica

C. Is prevented by the early introduction of cow's milk

D. Never require treatment under the age of 6 months

E. In childhood is associated with chronic blood loss in most cases

Ans:- B


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9. The following cause a microcytic anaemia:A. Ulcerative colitis.

B. Pernicious anaemia.

C. Methotrexate therapy.

D. Folate deficiency.

E. -Thalassaemia

Ans:-AE

10. Iron deficiency anaemia

A. May be prevented by the promotion of milk intake.B. Contribute to more significant anaemia in infants who are breast rather than formula fed for

the first 6 months.C. Is associated with higher incidence of early learning problems.

D. Is confirmed by demonstrating elevation of serum ferritin conc.

E. Increased susceptibility to infection.

Ans:-CE

Iron is absorbed 2 to 3 times more efficiently from human milk than from cow's milk. Breast-fed infants

may, therefore, require less iron from other foods.

11. The following are predisposing factors for iron deficiency anaemia:A. Drinking unmodified cow's milk

B. Prematurity

C. Infant of diabetic mother

D. Intake of bottle milkE. Excessive tea drinking

Ans:-ABE

Comments:

The predisposing factors/causes include:

Inadequate dietary intake of iron.Drinking unmodified cow's milk (doorstep milk).

Prematurity and low birth weight.

Food stuffs and beverages which reduce iron availability including tea.

12. Serum ferritin:A. Is raised in acute rheumatoid arthritis.

B. Stores 95% of the body's iron.

C. Is a useful measurement of iron storage in the body.

D. Should be measured in all cases of suspected iron deficiency.E. Is increased in hepatoma.

Ans:-ACComments:

Iron is an essential component in the structure of haemoglobin and myoglobin for oxygen and carbondioxide transport. It is also found in oxidative enzymes, cytochrome C and catalase. It is absorbed in the

ferrous form according to body need, aided by gastric juice and Vitamin C, and hindered by fibre, phytic

acid, and steatorrhoea (about 90% of intake is excreted in the stool). It is transported in the plasma in the

ferric state bound to transferrin, and is stored in the liver, spleen, bone marrow and kidney as ferritin and

haemosiderin. It is conserved and reused with minimal losses in the urine and sweat. In progressive iron

deficiency, a sequence of biochemical and haematological events occurs:

First: Tissue iron stores (bone marrow haemosiderin) disappear, and serum ferritin drops. Ferritin is a

relatively accurate estimate of body iron stores in the absence of inflammatory disease.

Next: Serum iron drops, and TIBC increases, and free erythrocyte protoporphyrins begin to accumulate.

Later: Red cells become smaller, and hypochromic with a drop in MCH and MCV. There may be

poikilocytosis and increased red cell distribution width. Reticulocyte count may be normal or

elevated, with nucleated red cells seen on the peripheral film. There may be thrombocytosis. The


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bone marrow is hypercellular with erythroid hyperplasia. Following iron therapy there is replacement of

intracellular iron enzymes and a subjective improvement within 24 hours. Within 48 hours there is a bone

marrow response, with reticulocytosis evident from 2 days, and peaking at about 7 days. The haemoglobin

level begins to increase from day 4 to day 30, and 3 months are required for complete repletion of iron

stores.

In most cases of suspected iron deficiency a low Hb plus microcytosis with response to iron therapy

obviates confirms the diagnosis, and there is no need to measure ferritin.

13. Regarding body iron stores:-A. serum ferritin is a poor guide to iron stores

B. a healthy young man will have more iron stored as ferritin than in the circulating red cell massC. iron is the stimulant for ferritin production

D. in iron deficiency stores of haemosiderin are mobilized before ferritin

E. if stained with nitroprusside stain both haemosiderin and transferrin have same colour

Ans:- C

14. Regarding iron deficiency anaemia:

A. The commonest cause in children is chronic blood loss.B. It does not affect school progress.

C. It commonly co-exists with thalassaemia.

D. Cow's milk is a major source of iron for children.

E. Lead poisoning is commonly associated with iron deficiency.

Ans:- E

Comments:Iron is absorbed in the proximal small intestine, mediated partly by the duodenal protein mobilferrin. About

10% of dietary iron is absorbed, and iron is absorbed 2-3 times more efficiently from human milk than frommodified cow's milk. During the first years of life, because relatively small quantities of iron-rich foods are

taken, it is often difficult to attain sufficiency iron. The diet should include foods such as infant

cereals or formulae that have been fortified with iron. Breast fed infants should receive iron supplementsfrom 4 months of age. At best, the infant is in a precarious situation with respect to iron. Should the diet

become inadequate, or external blood loss occur, anaemia ensues rapidly. In children with microcytic

anaemia who fail to respond to iron, thalassaemia should be considered. In this country there is an increased

incidence in those from the Mediterranean and those from the Indian sub-continent. Because many such

children are socioeconomically disadvantaged, there may be an associated iron deficiency anaemia. Lead

poisoning in this country is usually associated with eating lead paint. Since pica is associated with iron

deficiency, the two often co-exist.

15. In iron deficient anaemiaA. Hypochromia, microcytosis, and reduced red cell count are typically found.

B. Pencil cells may be seen in the blood filmC. Koilonychia and glossitis are common complications

D. Free erythrocyte protoporphyrin levels are elevatedE. Ingestion of cows milk before the age of 1 year is a recognized cause

Ans;-ABDE

16. regarding anaemia in childhood:-

A. Iron deficiency anaemia is the most common cause.

B. Mucosal pallor is useful clinical sign.

C. Macrocytic blood film indicates iron deficiency anaemia.

D. Blood transfusions are standard treatment.

E. Occur in less than 5% of population.

Ans :-AB

Iron deficiency is indeed common; in some population has been quoted as high as 30%.


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17. The commonest presentation in child with iron deficiency anemia:A. anaemia

B. Palpitation

C. usually asymptomatic

D. poor concentration

E. heart failure

Ans :-CD

18. Concerning sideroblastic anaemia:

A. Ring sideroblasts are seen in the peripheral blood film.B. Pancreatic exocrine dysfunction may occur.

C. It is associated with a low serum iron.D. It may occur with anti-tuberculous therapy.

E. Some cases may improve with pyridoxine.

Ans:-BE

Comments:The sideroblastic anaemias are a heterogeneous group of hypochromic, microcytic anaemias, probably due

to abnormalities of heme metabolism. Serum iron levels are raised, and ring sideroblasts are seen in the bonemarrow (nucleated red cells with perinuclear haemosiderin granules that represent iron-laden mitochondria).

Sub-types include:

Pearson Syndrome: associated exocrine pancreatic dysfunction due to deletions in mitochondrial DNA.

X-linked recessive: associated with splenomegaly. This may be pyridoxine sensitive or pyridoxine

refractory.

Acquired: various inflammatory and malignant processes, alcoholism.

19. The following are valid association in children with hypochromic anaemia:-A. Normal serum ferritin &an elevated HbA2 point to beta thalassemia minor

B. Low ferritin & normal HbA2 exclude beta thalassemia minor.

C. Low ferritin point to iron deficiency.D. Raised ferritin & normal hemoglobin electrophoresis point to anaemia of chronic

inflammatory disorder.

E. Dimorphic blood film is consistent with recent blood transfusion.

Ans:-ACDE

20. Which of the following statement regarding iron in infancy is false :-A. About 4% of iron in fortified cow milk formula is absorbed by the infants

B. About 10% of iron in unfortified cow milk formula is absorbed by the infants

C. About 15% of iron in breast milk is absorbed by the infants

D. Absorption of iron by the infant is generally greater than in adultsE. Cow's milk and human milk have approximately the same iron content

Ans:- C21. Hypochromic microcytic anaemia is seen in:

A. Alpha thalassemiaB. Sickle cell anemia

C. Autoimmune hemolytic anemia

D. Folate deficiency

E. Imerslund syndrome

Ans:- A

Comment:-Hypochromic microcytic anemia is usually associated with:

a) Fe deficiency

b) Sideroblastic anemia:

- Primary X-linked recessive

- Secondary alcohol

- Malignancy


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- Inflammation

In sideroblastic anemia there is Fe, but accumulation in the RBC mitochondria results in stippling on the

peripheral smear and ring sideroblast formation in the bone marrow.

Treatment is with pyridoxine (vitamin B6).

c) Lead poisoning

d) Beta-thalassemia (major or intermedia)

e) Alpha-thalassemiaf) Anemia of chronic disease

g) Copper deficiencySickle cell disease does not produce a microcytic anemia unless associated with thalassemia as in HbSb0.

To differentiate between Fe deficiency and beta-thalassemia use:

'Discriminant function'(DF) = MCV (fl) - RBC (x109/l) - (5 x Hb [g/dl]) - 3.4

If DF is +ve = Fe deficiency

If DF is -ve = beta-thalassemia trait

Beware; this is no use in hemodilution states (i.e. post hemorrhage or in pregnancy).

Megaloblastic anemia on the other hand is usually due to:

a) Folate deficiency

b) Vitamin B12 deficiency

c) Orotic aciduria

d) Pyruvate kinase deficiency

To discriminate between folate and B12 deficiency - think:

-Decreased RBC folate-Hyper-segmented neutrophils

-LDH

All suggest folate deficiency.

Imerslund syndrome is characterized by:

a) Chronic mucocutaneous candidiasis

b) Decreased PTH

c) Proteinuria

d) Decreased vitamin B12 absorption.

e) congenital autosomal recessive disorder which presents in the first 2 years of life as

megaloblastic anemia and failure to thrive.

22. Regarding anaemia in childhood:-

A. Hemoglobin concentration of 11g/dl at the age of 3 months merits investigation.B. Iron supplements should be given to all breast feeding infants for the first 6 months of life.

C. Serum iron is characteristically raised and iron binding capacity reduced in chronic hemolyticanemia.

D. The majority of cases of iron deficiency anaemia in the first 2 year of childhood are

nutritional in origin.

E. In the treatment of iron deficiency anaemia, parenteral iron therapy raises the hemoglobin

faster than oral iron.

Ans:-CD

23. In the anaemia of chronic infection:-

A. The hemoglobin is usually


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Ans:-CD

24. Anaemia of acute infection and chronic disease is associated with all the following except:-

A. Shortened red cell survival

B. Impaired iron utilization

C. Iron stores are usually reduced

D. Impaired erythropoietin and bone marrow response

E. Usually mild anaemia with normocytic or microcytic red cells in acute infectionAns:- C

25. Regarding blood indices:A. The haemoglobin concentration of a 3 month old boy is higher than a 13 year old boy.

B. The mean corpuscular haemoglobin is low in megaloblastic anaemias.C. The mean corpuscular haemoglobin concentration is low in iron deficiency anaemias.

D. The reticulocyte count increases with each year of life.

E. Reticulocytes are similar in size to mature red blood cells.

Ans:- C

Comments:

The haemoglobin at birth ranges from 13.7 - 21.1g/dl, falling to 13.0 - 20g/dl at 2 weeks of age, and tobetween 9.5 and 14.5g/dl at 3 months. From there it rises gradually, with normal ranges between 6 months

and 6 years being 10.5 - 14.0g/dl, and between 7 and 12 years being 11.0 - 16.0g/dl. Adult normal ranges for

females are12.0 - 16.0g/dl, and for males 14.0 - 18.0g/dl. The mean cell haemoglobin (MCH) is the

haemoglobin divided by the red cell count. In health, this is usually between 27 and 32pg. Any disorder

which reduces red cell size reduces the amount of haemoglobin in the cell, and lowers the MCH. Likewise,

disorders increasing cell size raise the value. Means corpuscular haemoglobin concentration (MCHC) equalsthe haemoglobin divided by the haematocrit. The normal range is 30 - 35g/dl. The MCHC is thus not the

number of grams of haemoglobin in 1dl of blood, but in 1dl of pure red cells without plasma. A low MCHCis usually due to iron deficiency. The reticulocyte count in cord blood is 5%, paralleling the change from Hb

F to Hb A. From 2 weeks of age it drops to 1%, and remains at this level until adulthood. Once menses start,

females have a slightly higher reticulocyte count of around 1.6%. Reticulocytes are slightly larger thanmature red cells because of the little remaining nuclear material.

26. Concerning vitamin B12:-A. Deficiency can cause ataxia

B. Pernicious anaemia commonly occur following terminal ileal resection

C. In deficiency MCV is usually normal

D. Deficiency usually occur in untreated celiac disease

E. Extrinsic factor promote absorption

Ans:- A

27. Juvenile pernicious anaemia is characterized by all of the following except:-

A. Gastric atrophyB. Concurrent endocrinopathy

C. Selective IgA deficiencyD. Autosomal recessive inheritance pattern

E. Chronic candidiasis

Ans;-

28. Abnormality associated with extravascular hemolysis include:-A. Increase in plasma hemoglobin

B. Decrease in serum haptoglobin

C. Increase in urinary hemosiderine

D. Increase in serum unconjugated bilirubin

E. None of the above

Ans:- D


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29. With respect to hereditary hemolytic anaemia:-A. Hereditary spherocytosis is the commonest hereditary hemolytic anaemia in Northern Europe.

B. Hereditary spherocytosis is confirmed by the presence of spherocytes on the blood smear and by

chromosome fragility test.

C. G6PD deficiency & pyruvate kinase deficiency are inherited in an autosomal recessive manner.

D. G6PD deficiency is confirmed by assaying the red cell G6PD enzymes.

E. The Embden-Meyhof pathway is unable to function in the red cell that is deficient in G6PD.Ans:-AD

30. Vitamine E deficiency is associated with all of the following except:-

A. Greater prevalence in small premature infants.B. Manifestation as anaemia with an elevated reticulocyte count.

C. Thrombocytosis.

D. Physical manifestation characteristic of this deficiency are edema of the legs , labia,and eyelids

in infants.

E. Result in an elevated alpha tocopherol level.

Ans:- E31. All of the following statements regarding hereditary spherocytosis are true except :A. Individuals with hereditary spherocytosis may be asymptomatic without anemia

B. The newborn with hereditary spherocytosis may present with anemia and .hperbilirubinemia

severe enough to require phototherapy and exchange transfusion

C. Isoimmune hemolytic anemia due to ABO incompatibility may mimic hereditary spherocytosis

D. Thermal injury can cause spherocytosisE. Splenectomy does not eliminate most of the hemolysis of hereditary spherocytosis

Ans:- EBecause the spherocytes in hereditary spherocytosis are destroyed almost exclusively in the spleen,

splenectomy eliminates most of the hemolysis associated with this disorder. After splenectomy, osmotic

fragility often improves because of diminished splenic conditioning and less RBC membrane loss, and theanemia, reticulocytosis, and hyperbilirubinemia resolve.

32. Feature of hereditary spherocytosisA. Splenomegaly

B. Conjugated hyperbilirubinemia

C. Gallstones

D. Hemolytic crises following fava bean ingestion

E. X linked inheritance

Ans:-AC

33. Hereditary spherocytosis:-

A. Is usually inherited in sex- linked recessive fashion.B. May necessitate exchange transfusion in the neonatal period.

C. Should routinely be treated by Splenectomy in infancy.D. Can't be diagnosed in asymptomatic patient.

E. May be complicated by gall stone.

Ans:-BE

34. Concerning hereditary spherocytosis:A. It is inherited as an autosomal dominant disorder.

B. The red cells have increased osmotic resistance.

C. Sufferers are prone to aplastic crises secondary to parvovirus infection.

D. It is associated with macrocytosis.

E. The cell shape returns to normal following a splenectomy.

Ans:-AC

Comments:


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Hereditary spherocytosis (HS) is a common cause of haemolysis and haemolytic anaemia, with a prevalence

of 1:5000 in Northern Europeans. Patients may be asymptomatic without anaemia and minimal haemolysis,

or have severe haemolysis. It is autosomal dominant, though it is occasionally transmitted as AR. 25% of

patients have no family history. The abnormality is in spectrin, a major component of the cytoskeleton. This

results in loss of membrane without a proportionate loss of volume, so the red cells end up as small spheres

rather than biconcave discs. There is an associated increase in cation permeability and transport, ATP

utilisation, and glycolytic metabolism. The cells have decreased deformability impairing splenic passage, sothe cells are prematurely destroyed. In addition to haemolysis, hypoplastic crises may be associated with

parvovirus infection. This may result in profoun anaemia with high output heart failure, hypoxia,cardiovascular collapse and death. In HS the haemoglobin level is usually 6 g/dl, and the reticulocyte count

is elevated to 6-20%. The MCV is normal, but the MCHC is increased. Spherocytes have a smaller diameterthan normal cells. The diagnosis is confirmed by an osmotic fragility test. Splenectomy eliminates most of

the haemolysis associated with the disorder, but carries its own risks. In mild cases, folic acid 1mg/day is

administered to prevent secondary folate deficiency. For those with severe anaemia, and/or hypoplastic or

aplastic crises, splenectomy is recommended after the age of 5-6 years. Vaccines for pneumococcus,

meningococcus and haemophilus influenzae should be given prior to splenectomy, and prophylactic

penicillin continued for life.

35. In hereditary spherocytosis ,all of the following are characteristic except:-A. It is autosomal dominant trait

B. There is loss of red cell membrane surface without reduction of cell volume ,necessitating

spherical shape in order to accommodate it's content

C. Spherocytes are more likely to rupture than normal cells when suspended in hypotonic solutionD. The MCV is increased & the MCHC is decreased

E. Clinical severity tend to be relatively consistent within families although it varies widely fromfamily to family

Ans:-D

36. Hereditary spherocytosis :-A. Is inherited as an autosomal dominant condition.

B. May be confirmed by paper electrophoresis of whole blood.

C. May cause sever jaundice in the newborn period.

D. May be complicated by abrupt exacerbation of anaemia due to transient erythroid hyperplasia

E. Is an indication for Splenectomy in the first 3 year of life.

Ans:-ACD

37. Concerning hereditary spherocytosis:-A. Presence of spherocytes in the neonatal period is diagnostic of this condition .

B. Inheritance is as an autosomal dominant .C. The direct coomb's test is positive in > 50% of cases.

D. The condition may present as jaundice in the neonatal period.E. Splenectomy reverses the tendency to form spherocytes.

Ans:-BD

38. In child presenting with acute hemolysis due to an inherited hemolytic anaemia:-

A. Anaemia is present only after complete failure of haemtopoiesis.

B. The plasma conjugated bilirubin level is elevated.

C. Reticulocyte count will be less than 1%.

D. Plasma haptoglobin will be elevated.

E. There is excess urinary urobilinogen.

Ans;- E

39. Characteristic of G6PD deficiency in patient include all of the following except:-A. Sex linked recessive patter of inheritance

B. Persistence of low grade anaemia in patient

C. The most common clinical manifestation is episodic acute hemolysis usually follow infection or

drug ingestion


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D. In black G6PD activity is near normal in reticulocytes and young erythrocytes

E. Heinz bodies may be present only early in hemolytic episodes

Ans:- B

40. Glucose-6-phosphate dehydrogenase deficiency:

A. Is inherited as a sex-linked condition

B. is not clinically manifest in girls

C. Causes drug-induced haemolysisD. Is an indication for Splenectomy

E. Is more pronounced in mature red blood cellsAns:-ACE

Comments:G6PDH deficiency is x linked recessive. It can manifest in females if there are 2 copies of the abnormal

gene. Drugs which cause haemolysis include aspirin, antimalarials, antibacterials and sulphonamides.

Splenectomy is not helpful in the management of this condition. Young red blood cells have near

normal enzymatic capacity.

41. All of the following statements regarding G6PD deficiency are true except :-

A. Symptoms usually develop in patients with G6PD deficiency 24-48 hr after ingesting a

substance with oxidative properties

B. Infection may result in hemolysis in patients with G6PD deficiencyC. A pregnant woman who ingests oxidative drugs may cause hemolytic anemia in a fetus with

G6PD deficiencyD. Enzyme activity in affected persons is 10% of normal or less

E. The usual dose of aspirin causes clinically relevant hemolysis in the A variety of G6PDdeficiency

Ans:- E

The usual doses of aspirin and trimethoprim sulfamethoxazole do not cause clinically relevant

hemolysis in the A- variety. However, aspirin administered in doses used for acute rheumatic fever (60100 mg/kg/24?hr) may produce a severe hemolytic episode. When hemolysis has occurred, supportive

therapy may require blood transfusions, although recovery is the rule when the oxidant agent is

removed.

42. All of the following statements about Glucose-6-phosphate dehydrogenase deficiency are false

except:A. Is more common in women than in men.

B. Is caused by mutations of the G6PD gene on chromosome 4.C. Often leads to chronic haemolysis.

D. Requires treatment with desferrioxamine to prevent iron overload.

E. Is associated with haemolytic crises induced by sulfonamides.Ans:- E

G6PDH catalyses the synthesis of NADPH from the hexose monophosphate pathway. The NADPH is then

used to reduce glutathione and hence in conjunction with glutathione reductase reduces cellular oxidative

damage.

G6PDH enzyme deficiency, caused by mutations in the X-linked (Xq28) G6PD gene, affects over half a

billion people worldwide. It is a balanced polymorphism associated with resistance to falciparum malaria in

heterozygous females. This evolutionary advantage outweighs the small negative effect of affected

hemizygous males. At least 400 G6PD variants have been identified. The various mutations lead to altered

expression of G6PD or an altered half-life of the enzyme.

There are a number of disease manifestations:

- patients may be asymptomatic- patients may present with neonatal jaundice

- usually the deficiency manifests as acute haemolytic crises provoked by oxidising stress e.g.certain foods - fava bean, chemicals - naphthalene, certain drugs - dapsone, primaquine, chloroquine, aspirin


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(high doses), sulphonamides, nitrofurantoin, vitamin K and nalidixic acid.

Infections can also provoke haemolysis. Haemolytic crises vary in severity, rarely chronic haemolysis may

occur. If severe, G6PD deficiency can cause immunodeficiency by reducing the NADPH required for

activation of the NADPH oxidase enzyme (cf. CGD).

43. Chronic intravascular haemolysis is associated with:

A. Splenomegaly.B. Haemosiderinuria.

C. Increased incidence of gallstones..D. Reduced transferrin levels.

E. Increased serum haptoglobins.Ans:-BC

Comments:Haemolysis is defined as the premature destruction of red cells. If the rate of destruction exceeds the

capacity of the marrow to produce red cells then anaemia results. The normal red cell survival is 120 days,

and 1% of red cells are removed per day and replaced by the marrow. During haemolysis, the red cell

survival is shortened, and there is increased marrow activity (raised reticulocyte count). Marrow output canincrease 2-3 fold acutely, and 6-8 fold in long-standing cases. In chronic haemolytic anaemia, erythroid

hyperplasia may be so extensive that the medullary spaces may expand at the expense of cortical bones

(particularly skull and long bones). Intravascular haemolysis increases circulating haemoglobin which binds

to haptoglobin reducing the circulating levels of it. There is an increase in urine haemoglobin and

haemosiderin. Increased bilirubin production from heme results in increased faecal urobilinogin, which is

reabsorbed and excreted in the urine.

44. The following drugs should be avoided in all patients with glucose-6-phosphate dehydrogenase

deficiency:

A. Quinine

B. NitrofurantoinC. Sulphapyridine

D. Chloramphenicol

E. Chloral hydrate

Ans:- ABCD

Comments:In glucose-6-phosphate-dehydrogenase deficiency (G-6-PD) subjects are susceptible to developing acute

haemolytic anaemia on taking a number of common drugs. Ingestion of fava beans may result in haemolysis

in severe cases, or when they are eaten raw. G-6-PD is genetically heterogeneous. The risk from drugs

therefore varies from patient to patient. There is no test available to identify potential risk in G-6-PDdeficiency. The risk of severity of haemolysis is almost always dose related. Drugs with a definite risk in

most G-6-PD deficient subjects include: Sulphonamides and Dapsone.

Methylene blue Nitrofurantoin

Primiquine

Quinilones (including Ciprafloxacin, Nalidixic acid)

45. The following are associated with a definite risk of hemolysis in most glucose-6 phosphate

dehydrogenase deficient subjects:A. Primaquine

B. Methyllene blue

C. Nalidixic acid

D. Co-trimaxazole

E. Nitrofurantoin

Ans:- ABCDE


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glucose-6 phosphate dehydrogenase reduces NADP to NADPH. NADPH is used by glutathione reductase

to reduce (oxidised) glutathion (toGSH). This replenishes the supply of reduced-SH groups in the

erythrocyte membrane protecting it from oxidant stress.

Deficiency of G6PD renders the red cell membrane susceptible to oxidant stress and haemolysis may result

following exposure to an oxidising agent of the environment (drugs, fava beans, infections, DKA).

G6PD also reduces NAD to NADH which is used by methaemoglobin reductase. Therefore G6PDdeficiency may also result in increased oxidation of Hb (metHb) with the apperarance of Heinz bodies on

the blood film. Drugs that cause hemolysis in G6PD deficiency are oxidizing agent. The BNF gives two lists

of drugs as follows:

(1)- Definite risk of hemolysis in most: 2)-Possible risk in some:

*dapson. *aspirin (high dose).*methylene blue. *chloroquine.

*nitrofurantoin. *menadione (vit K).

*Primaquine. * probencid.

*quinolones *quinidine,.

*sulphonamides. *quinine

46. G6PD deficiency:-

A. Is inherited as sex-linked condition.

B. Is not clinically manifest in girls

C. Causes drug induced hemolysis.

D. Is an indication for Splenectomy.

E. Is more pronounced in mature RBCAns:-ACE

Most male show complete expression but female less so; American black the prevalence in male is 15% &in female is 2%

47. Regarding glucose-6-phosphate dehydrogenase:

A. It is an important enzyme in the kreb cycle.

B. Deficiency is associated with an increased susceptibility to falciparum malaria infection.

C. Deficiency may be associated with chronic haemolytic anaemia.

D. Deficiency is inherited as an autosomal dominant.

E. Deficiency may be associated with primiquine-induced haemolysis.

Ans:- E

Comments:Glucose-6-phosphase dehydrognease (G-6-PD) is one of the enzymes in the pentose phosphate pathway

(Hexose Monophosphate Shunt). The most important function of this pathway is to maintain glutathione in a

reduced state as protection against oxidation of the red cell. Deficiency is X-linked, and extremely common,affecting more than 200 million people. Heterozygous females have increased resistance to falciparummalaria, outweighing the small negative effect on hemizygous males. Major symptoms include:

Episodic or induced haemolytic anaemia: there is considerable variation in the defect among differentracial groups, with Mediterranean whites affected more severely than Africans. Haemolysis may precipitated

by:1. Drugs: Antibacterials: sulfonamides, septrin, nalidixic acid, chloramphenicol, nitrofurantoin.

Antimalarials: primaquine, chloroquine, quinacrine. Others: Vitamin K, methylene blue, Aspirin.

2. Chemicals: Phenylhydrazine, benzene, naphthalene.

3. Illness: Diabetic ketoacidosis (DKA), hepatitis.

Chronic haemolytic anaemia: chronic non-spherocytic haemolytic anaemia is associated with profound

deficiency of G-6-PD. Splenectomy is of little value in these types.


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48. Regarding autoimmune hemolytic anaemia (AIHA):-A. The direct coombs test will be positive.

B. It is frequently due to an intercurrent viral infection in children.

C. Spherocytes are commonly seen on the blood smear.

D. AIHA is divided into warm & cold types depending on whether the patient is febrile or afebrile.

E. It is often associated with connective tissue disorders in the adolescent.

Ans:-ABCE

49. A 17 year old Nigerian presents with moderately enlarged spleen, haemoglobin of 7.2 g/dL, a red

cell count of 3.5 x 1012/L, and a reticulocyte count of 7%. The following statements are correct:A. Recent chloramphenicol administration would be of diagnostic significance

B. If the Coombs' test was positive, paroxysmal nocturnal haemoglobinuria is possiblediagnosis

C. Homozygous thalassaemia is possible diagnosis

D. If Heinz bodies are present, red cell glucose 6-phosphate dehydrogenase deficiency is

likely diagnosis

E. homozygous sickle cell disease is likely diagnosis.

Ans:-C DComments:b-thalassaemia major (homozygous) thalassaemia is possible diagnosis. Splenic atrophy from splenic

infarcts occurs in sickle cell disease. Chloramphenicol may cause aplastic anaemia - there will be no

reticulocyte response. Heinz bodies are oxidized denatured haemoglobin. During crisis of G6PD deficiency,

blood film also shows contracted and fragmented cells due to oxidant stress. PNH is not an autoimmune

haemolytic anaemia, hence Coombs test is negative. It is a rare acquired defect of red cell membrane makingit susceptible to lysis by complement.

50. Features which may be seen on the blood film of a patient with haemolysis include:

A. Polychromasia

B. Howell Jolly bodiesC. Elliptocytes

D. Target cells

E. Heinz bodies

Ans:-ABDE

Comments:The following are common abnormalities of red cell morphology:

Polychromasia: younger cells have a bluish tinge (basophilia), and are larger than average, and may

contain residual nuclear material (reticulocytes). The presence of many basophilic forms in a blood film

produces a multicoloured effect known as polychromasia. Microcytosis indicates small red cells, anisocytosis variation in size, and macrocytosis a large size.

Poikilocytosis indicates altered shape. Spherocytes may indicate hereditary spherocytosis, and may also be found in acquired haemolytic

anaemias, while elliptocytes are found in hereditary elliptocytosis. Schistocytes are fragments of red cells, as seen in DIC or haemolytic uraemic syndrome.

In splenic dysfunction: target cells and red cells containing nuclear fragments known as Howell Jolly

bodies are seen. Pappenheimer bodies are iron containing inclusions, and when seen together with Howell

Jolly bodies suggest previous splenectomy or reduced splenic function.

Heinz bodies: are denatured haemoglobin which result from deficiencies of enzymes of the penthose

phosphate pathway. This damages the red cell leading to haemolysis and removal of them by the spleen.

The following conditions are haemaglobinopathies:-

A. G6PD deficiency.

B. von Willebrand disease.

C. Spherocytosis.

D. Thalassaemia..


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E. Sickle cell disease.

Ans:-DE

51. Homozygous beta thalassemiaA. Arise from alteration in the beta globin gene on chromosome 10 .

B. May present with FTT.

C. Is associated with elevated HbF in infancy & older children.

D. May require Desferoxamine treatment.E. Doesn't responds to iron treatment despite microcytic indices.

Ans:-BCDE

52. Which is compatible with B-thalassemia major?

A. HbF 96% HbA2 4%B. Hb A1 40%, Hb F 58%, Hb A2 2% .

C. Hb A1 85%, Hb F 10%, HbA2 5%

Ans: A

53. In beta thalassaemia:-A. Beta thalassaemia major presents at birth with profound anaemia

B. The reticulocyte count is lowC. Diagnosis can be made by Haemoglobin electrophoresis

D. Anaemic patients should receive regular iron supplementation

E. Splenectomy is contraindicated due to extramedullary haemopoiesis

Ans:- C

Beta thalassemia syndromes are a group of hereditary disorders characterized by a genetic deficiency in

the synthesis of beta-globin chains. In the homozygous state, beta thalassemia (ie, thalassemia major)causes severe transfusion-dependent anemia with high reticulocyte count.

The anaemia becomes manifest in late infancy. Diagnosis is by haemoglobin electrophoresis.Increased iron deposition resulting from multiple life-long transfusions and enhanced iron absorption

results in secondary iron overload. This overload causes clinical problems similar to those observed with

primary hemachromatosis (eg, endocrine dysfunction, liver dysfunction, cardiac dysfunction)o The physical findings are related to severe anemia, ineffective erythropoiesis, extramedullary

hematopoiesis, and iron overload resulting from transfusion and increased iron absorption.

o Skin may show pallor from anemia and jaundice from hyperbilirubinemia.

o The skull and other bones may be deformed secondary to erythroid hyperplasia with intramedullary

expansion and cortical bone thinning.

o Heart examination may reveal findings of cardiac failure and arrhythmia, related to either

severe anemia or iron overload.

o Abdominal examination may reveal changes in the liver, gall bladder, and spleen.

Hepatomegaly related to significant extramedullary hematopoiesis typically is observed.

Patients who have received blood transfusions may have hepatomegaly or chronic hepatitis due to ironoverload; transfusion-associated viral hepatitis resulting in cirrhosis or portal hypertension also may be

seen. The gall bladder may contain bilirubin stones formed as a result of the patient's life-long hemolyticstate. Splenomegaly typically is observed as part of the extramedullary hematopoiesis or as a

hypertrophic response related to the extravascular hemolysis.o Extremities may demonstrate skin ulceration.

o Iron overload also may cause endocrine dysfunction, especially affecting the pancreas,testes& thyroid.

54. In thalassemia:-A. The diagnosis is made on blood film.

B. The anaemia is due to deficiency in synthesis of globin chain.

C. The anaemia is mainly due to hemolysis.

D. Splenectomy is the treatment of choice at diagnosis.

E. The serum iron level is normal at diagnosis.

Ans;- BE


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55. The clinical picture of B-thalassemia major:-A. Present at birth.

B. Is characterized by iron deficiency anaemia.

C. May initially present as FTT

D. Include hepatosplenomegaly.

E. Is confirmed if investigation reveals markedly raised HbF on electrophoresis.

Ans:- CDE

56. Beta thalassemia major:-

A. Is associated with relative excess ofb globin molecules.

B. Result in blood film exhibiting microcytic hypochromic red cell.

C. Produce generalized osteoporosis.

D. Is commonly associated with reduced serum iron.

E. Result in delayed puberty.

Ans:- BCE

57. The following are characteristic finding in beta thalassemia :-A. Hypochromic microcytic red cells

B. Low serum ironC. Cardiomyopathy.

D. Presentation in the neonatal period.E. Frontal bossing of the skull.

Ans:-ACE

58. Thalasemia major is associated with all of the following except:-

A. Defect in globin chain synthesis

B. In the newborn infant is clinically & hematologically normal

C. The determination of HbA2 is important in diagnosis

D. Onset of clinical symptoms & sign usually begin between 6-12 month of age

E. Peripheral blood show hypochromic and usually microcytic anaemia

Ans;- C59. Which one of the following doesn't characterize heterozygote thalassemia ( minor / trait):-

A. In most patient anaemia & clinical symptoms are absent

B. Life expectancy is shortened

C. Varieties of this disorder are best distinguished by quantification of A2 & HbF

D. Peripheral blood examination reveal microcytosis ,target cells , & variable degree ofhypochromia

E. Severity depend on the degree of suppression of globin synthesisAns:- B

60. All of the following statements are true Except:A. In patients who have Hb SS electrophoresis pattern and concomitant microcytosis, iron deficiency or

a combination of Hb S with or thalassemia must be considered a possible diagnosisB. Febrile infants with sickle cell disease should be managed in the hospital

C. The iron in hemoglobin is normally in the ferric state, which is essential for oxygen transport

D. Children with homozygous thalassemia usually become symptomatic from progressive hemolytic

anemia during the second 6 mo of life if not treated

E. The thalassemia traits present as microcytic anemia, which can be mistaken for iron-deficiency

anemia

Ans:- C

61. A black West African girl aged 15 years is complaining of severe pain in both legs. The

haemoglobin is 8g/dl. Sickle cell anaemia is unlikely to be the cause of her symptoms if

A. she is jaundicedB. she has haematuria

C. x-ray of the spine shows osteoporosisD. menarche occured at 12 years

E. the urinary osmolality is 800mosm/kg (specific gravity approximately 1022)


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Ans:- DE

Comments:

a+b-expected in sickle cell anaemia, c-x-ray usually shows fishmouth vertebrae (infarcts), d-growth +

development usually impaired, e-almost all are unable to produce concentrated urine

62. In the management of acute sickle cell painful crises:

A. Patients should receive continuous oxygen and monitoring of SaO2.B. Patients should receive intravenous antibiotics.

C. Intravenous opiate analgesia should be titrated until adequate control of pain.D. Blood transfusions should be given.

E. Intravenous hydroxyurea should be given.Ans:-ABC

Comments:Painful episodes can often be managed with oral Paracetamol -/+ codeine. Severe episodes require

hospitalisation with parenteral opiates. Anti-inflammatories may decrease or eliminate the need for

narcotics. Dehydration and/or acidosis should be rapidly corrected by intravenous fluids. Packed cells are

specifically indicated for acute splenic sequestration and aplastic crises. The latter may require splenectomy.Intravenous antibiotics should be given to cover the possibility of haemophilus or pneumococcal infection.

Chemotherapy regimens that stimulate fetal haemoglobin synthesis have been employed with beneficial

effect on an experimental basis. These include hydroxyurea and hydroxybutyrate. These are given as

maintenance therapy.

63. The following are recognised features of sickle cell disease:A. Dactylitis

B. Frontal bossing of the skullC. Abdominal pain

D. Chronic leg ulceration

E. Anaemia from birthAns:- ACD

Comments:

Sickle cell anaemia is characterised by severe chronic haemolytic disease resulting from premature

destruction of brittle, poorly deformable erythrocytes. Other manifestations are due to ischaemia resulting

from vascular occlusion by masses of sickle cells. The clinical course is typically associated with crises. The

manifestations vary considerably with age.

Newborns: haemolytic anaemia from 2-4 months as fetal haemoglobin is replaced by Hb S, acute sickle

dactylitis (hand-foot syndrome).

Pre-school: acute painful vaso-occlusion crises, affecting extremities.School children: painful crises affecting head, chest, abdomen, back, the site being typical for an any

individual patient. Episodes may be precipitated by intercurrent illness.Late changes:

- infarction of bone marrow or bone.- splenic infarction between 6 and 60 months contributing to autosplenectomy.

- pulmonary infarcts (acute chest syndrome).

- stroke caused by cerebrovascular occlusion -/+ hemiplegia.

- ischaemic damage to myocardium, liver and kidneys, with progressive impairment of renal function

and concentrating ability.

Spleen changes: in young children the spleen is enlarged, with occasional acute splenic sequestration.

Altered splenic function increases the risk of serious infection particularly meningitis sepsis caused by

pneumococci and haemophilus influenzae (polysaccharide encapsulated organisms). As the child

ages, auto splenectomy reduces spleen size.

Cardiomegaly is invariably present in older children (sickle-related cardiomyopathy), secondary

haemosiderosis from increased iron absorption may damage liver, pancreas and heart, and there may be gall

stone formation. Puberty is frequently delayed, and chronic leg ulcers occur in late adolescence.


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64. A healthy, 39-week gestation male weighing 3.5 kg is born to a mother who has chronic anemia.

The infants physical examination findings are normal, and his hematocrit is 49% (0.49). The

mother asks you about the need for vitamins and iron for her newborn son.

Of the following, the BEST response is that term babies need to begin iron therapy atA. birth

B. 2 weeks of ageC. 4 months of age

D. 9 months of ageE. no time

Preferred Response: CTerm newborns have accrued sufficient iron stores in the latter part of gestation to sustain them for 3 to 4

months after birth; this is true even when the mother has anemia. Although human milk contains lower

quantities of iron, its bioavailability is greater and, therefore, breastfed infants do not require replacement

therapy until 4 months of age. Preterm infants miss out on iron accretion in utero during the last trimester of

pregnancy and may require iron supplementation if they are taking full-volume enteral feedings as early as 2

to 4 weeks of age.Iron supplementation is not required at birth except in the rare circumstance of congenital anemia. Iron

supplementation is required for normal hematopoiesis and brain growth and function, and if not provided by

6 months of age, characteristically leads to iron deficiency anemia.

65. You are called to the delivery room to evaluate an infant who has been delivered by spontaneous

vaginal delivery. The term infant weighs 3.6 kg and has some grunting respirations. You decide to

observe her in the newborn intensive care unit. One hour later, you are notified that the infant is

experiencing pronounced respiratory distress and oxygen saturations in the 70% range in the

right hand and in the 50% range in the right foot. There is poor perfusion. There are no murmurs,

but there is a pronounced precordial lift and a loud second heart sound.

Of the following, the MOST likely diagnosis for this infant isA. congenital diaphragmatic hernia

B. congenital toxoplasmosis

C. hypoplastic left heart syndrome

D. persistence of the fetal circulation

E. tetralogy of Fallot

Preferred Response: D

Determining the cause of cyanosis and hypoxemia in the neonate is critically important, but it can be

difficult, especially during the first few minutes of the presentation. Diagnostic considerations in these

potentially critically ill infants may include persistence of the fetal circulation, with or without meconium

aspiration, and cyanotic congenital heart disease. The neonate described in the vignette clearly is

experiencing a disturbance in the process of oxygen delivery, and several clues suggest persistence of thefetal circulation. Detecting this condition requires a clear understanding of the ductus arteriosus and its

function in the transitional circulation.In the normal heart, the right atrium and right ventricle deliver desaturated blood to the organ of

oxygenation. In the fetus, this organ is the placenta, and its fetal blood supply is via the umbilical artery,which arises from the fetal descending aorta. The ductus arteriosus provides a fetal shunting pathway,

allowing the right side of the heart to deliver desaturated blood to the placenta by shunting this blood away

from the high-resistance pulmonary arteries and into the descending aorta. This direction of flow is due in

part to the slightly higher fetal pulmonary vascular resistance compared with fetal systemic vascular

resistance because the lungs are filled with fluid and the placenta is a low-resistance circuit.

At birth, when the lungs expand with air and the placenta is removed from the circulation, pulmonary

vascular resistance falls and systemic vascular resistance increases. This leads to a reversal of flow across

the ductus arteriosus (from the system into the pulmonary circuit). Over

subsequent hours and days, the ductus arteriosus begins the process of spontaneous closure.


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When the pulmonary vascular resistance exceeds the systemic vascular resistance, right-to-left shunting

across the ductus arteriosus persists as long as the ductus arteriosus is patent. Neonates who have severe

lung disease, including pneumonia, lung collapse, and pulmonary hypoplasia, may have pulmonary vascular

resistance that exceeds the systemic vascular resistance. When this occurs in the presence of a patent ductus

arteriosus, there is right-to-left shunting of blood in a pattern that is similar to the fetal circulation. Hence,

the term persistence of the fetal circulation, or perhaps better stated, persistent pulmonary hypertension

of the newborn. This condition leads to highly desaturated blood entering the descending aorta, oftenevidenced by lower oxygen saturation in the lower body than in the upper body. When this process results

from pulmonary pathology, there is likely intrapulmonary ventilation-perfusion (V-Q) mismatch as well,yielding the return of relatively desaturated blood to the left heart, which then delivers inadequately

oxygenated blood to the aorta.Signs of an underlying pulmonary process may include grunting to maintain alveoli and small airway

patency, tachypnea, and ultimately respiratory distress and failure, as described for the infant in the vignette.

The oxygenation saturation pattern of the infant supports the diagnosis of persistence of the fetal circulation.

She has no murmur because she has no turbulent blood flow in her heart, and her precordial lift results from

the increased volume, pressure, and work load imposed on the right ventricle. Poor perfusion is caused by

failure (diminished ventricular function) of the right ventricle and its impact on the left ventricle.Neonates who have diaphragmatic hernia often present with respiratory distress and a shift of the

precordium to the right chest because the gastrointestinal contents typically occupy the left thorax.

Infants who have toxoplasmosis may have signs and symptoms of systemic infection, but they are

unlikely to present solely with pulmonary pathology.

The hypoplastic left heart syndrome relies on right-to-left shunting at the ductus arteriosus to supply

systemic blood flow, but affected infants have normal lungs and low pulmonary vascular resistance, withoxygen saturation typically in the 80% to 90% range.

Tetralogy of Fallot usually presents with the murmur of pulmonary stenosis, and when the ductusarteriosus is present, blood flow is left to right because pulmonary vascular resistance and the lungs are

normal

66. In sickle cell disease:-A. Dactylitis is common presentation in infancy.

B. Autosplenectomy has generally occurred by the age of 6 months.

C. Sickle lung is an indication for exchange transfusion.

D. Aplastic crises may arise as consequence of parvovirus infection.

E. Salmonella osteomyelitis occur.

Ans:-ACDE

Dactylitis, often referred to as hand-foot syndrome, is frequently the 1st manifestation of pain in children

with sickle cell anemia, occurring in 50% of children by 2 yr of age. Dactylitis often presents withsymmetric swelling of the hands and/or feet

Acute splenic sequestration is a life-threatening complication occurring primarily in infants, and may occuras early as 5 wk of age. Approximately 30% of children with sickle cell anemia have significant splenic

sequestration episodes; Repeated episodes of splenic sequestration are common, occurring in approximately50% of patients. The majority of recurrent events occur within 6 mo of the previous event.

67. Sickle cell disease :-

A. Is caused by a mutation on chromosome 16

B. Will give a positive sickle test result at birth

C. Is associated with strokes in 25% of patients by the age of 45 years

D. Is an indication for autologous haemopoietic stem cell transplant

E. Can be co-inherited with alpha or beta-thalassaemia

Ans:- CE

Sickle cell disease is an autosomal recessive condition caused by a single amino acid mutation in the Beta

globin gene on chromosome 11. The alpha globin genes are coded on chromosome 16. It can be co-inherited

with alpha or beta thalassaemia. Clinical effects do not become apparent until 6 to 12 months of age when


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haemoglobin switching occurs and the amount of HbF (fetal haemoglobin) decreases and the amount of HbS

(sickle haemoglobin) increases. This is why the sickle test, which detects the presence of sickle

haemoglobin, is often not positive until 6-9 months of age and cannot be guaranteed until one year of age.

Clinical problems include strokes and these are common, occuring in 25% of patients with homozygous

sickle disease by the age of 45 years. Patients with severe clinical manifestations of sickle disease have been

treated with allogeneic bone marrow transplantation from an HLA-identical sibling. Autologous bone

marrow transplantation is where the patients own bone marrow is replaced and would not be of benefit inthis condition.

68. Sickle cell disease :-

A. Is associated with increased the polymerization of deoxygenated HbB. Is recognized cause of prolonged neonatal jaundice.

C. Increased the susceptibility to infection with encapsulated bacteria.

D. Is cause of childhood stroke ( cerebrovascular disease ).

E. Is conventionally managed by regular blood transfusion.

Ans:-ACD

69. In sickle cell disease:-A. There is abnormality of red cell membrane.

B. Symptoms result from tissue infarction.

C. There is some protection against Plasmodium falciparum malaria.

D. Sickling crises can be prevented by morphine.

E. Sickling risk can be reduced by exchange transfusion.

Ans:-BEIndividuals with sickle cell trait have lower levels of Plasmodium falciparum parasitemia, higher

hemoglobin counts, and less severe reinfections than individuals with homozygous Hb A.

70. Regarding sickle cell anaemia :-

A. Positive Sickling test is diagnostic of sickle cell diseaseB. Roughly 5% of African are carriers of the sickle cell trait.

C. The heterozygous condition is usually asymptomatic.

D. Treatment with desferoxamine has significantly improved the life expectancy in sickle cell

disease.

E. There is increased predisposition to salmonella osteomyelitis.

Ans:-CE

the incidence ofsickle cell trait is 710% of African-Americans (1 in 12. ). The amount of Hb S in

individuals with sickle cell trait is


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It is not possible for the baby to have sickle cell disease (HbSS).

72. Sickle cell anaemia:-A. Is associated with increased risk of gallstone.

B. Is associated with increased risk of nocturnal enuresis

C. Can be diagnosed antenatally

D. Is an indication for prophylactic penicillinE. Is associated with episodes of acute anaemia

Ans:-ABCDE

73. Complications of sickle cell disease include:

A. Pneumococcal infection in the first four weeks of life.B. Stroke.

C. Dactylitis in the first four weeks of life.

D. Cholecystitis.

E. Haematuria.

Ans:-BDE

Dactylitis (swelling of the joints)is the most common presentation symptom of sickle cell disease. It usuallyoccurs between six to twelve months of age when haemoglobin production switches from the production of

gamma chains to the production of the abnormal beta chains, and may present to an orthopaedic surgeon.

This means that the amount of foetal haemoglobin (which is protective against sickle crisis)decreases and

the amount of sickle haemoglobin increases.

Pneumococcal infections (and infections with other encapsulated bacteria such as Haemophilus influenza B

and neisseria meningitides) are common in patients with sickle cell disease from the age of 2 to 3 yearswhen they have auto-infection of the spleen and are rendered hyposplenic. They should be protected against

these diseases with vaccination and prophylactic antibiotics.Strokes are very common in patients with sickle cell disease occurring in 25% of patients with homozygous

sickle cell disease by the age of 45 years.

Cholecystitis occurs in patients with sickle cell disease as with a haemolytic anaemia because of theproduction of pigment gallstones.

Haematuria occurs in sickle cell disease because of renal papillary necrosis. Interestingly it can also occur in

patients with sickle cell trait.

74. Which of the following statement is false regarding the pathophysiology of sickle cell disease:-A. Infection is associated with increased anemia by suppression of RBC production

B. Conversion of RBC from normal biconcave discs to sickle form require the deoxygenation of

haemoglobin

C. Hypoxiemia and acidemia promote sickling by decreasing oxygen saturation of HbD. Dehydration doesn't promote sickling

E. Sickle cell increase whole blood viscosity producing local ischemiaAns:-D

75. Which one of the following clinical manifestation isn't common to sickle cell disease:-A. Vaso-occlusive crisis is more common than aplastic crisis

B. Hyperhemolytic crisis is often associated with red cell G6PD deficiency

C. Aplastic crisis is often associated with viral or bacterial infection

D. Salmonella sepsis is commonly associated with salmonella osteomyelitis

E. Hand-Foot syndrome may be initial manifestation of sickle cell disease

Ans:-D

76. The following are seen in sickle-cell anaemia

A. dactylitis

B. retardation of secondary sexual characteristics.

C. pathognomic fundal changes

D. cardiac signs simulating mitral stenosis

E. normal urinary concentrating ability only if the sickle-cell trait is present


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Ans:-ABCD

Comments:

Dactylitis - inflammation of the fingers is a feature of SCD due to expansion of bone marrow. B -

aconsequnce of ill health with hypogonadotrophic hypogonadism. A so called black sunburst retinopathy is

pathognomonic of sickle cell disease. Retinal vein thrmbosis is also seen. Poor urinary concentration is a

feature of the disease but is unaffected with the trait.

77. Recognised features of sickle cell trait include:

A. Moderate anaemiaB. Increased risk of anaesthesia

C. Reduced renal concentrating ability in adolescenceD. Episodic haematuria

E. Splenomegaly

Ans:-BCD

Comments:Heterozygous expression of the sickle haemoglobin gene (Hb AS) is usually associated with a totally benign

clinical course. The haematological findings are indistinguishable from normal. About 40% of theirhaemoglobin consists of Hb S, and under normal circumstances this is insufficient to produce sickling.

Under severe hypoxic stress, vaso-occlusive complications may occur. This may occur under anaesthesia or

at high altitudes. This may result in splenic infarcts and other ischaemic sequelae. Decreased renal

concentrating ability is usually present in older children and adults, and occasional gross haematuria may

occur. The diagnosis is confirmed by haemoglobin electrophoresis and sickle testing.

78. A 10 year old West African boy presents with Hb 8g/dl and pains in his legs. Sickle cell disease is

unlikely if:A. He is jaundiced.

B. He has gross splenomegaly.

C. Puberty began at 12 years.D. His urine osmolality is 800mosmol/L, specific gravity 1022.

E. There is a mid systolic murmur.

Ans:-BCD

Comments:

Sickle cell anaemia is characterised by severe chronic haemolytic disease resulting from premature

destruction of brittle, poorly deformable erythrocytes. Other manifestations are due to ischaemia resulting

from vascular occlusion by masses of sickle cells. The clinical course is typically associated with crises.

The manifestations vary considerably with age.

Newborns: haemolytic anaemia from 2-4 months as fetal haemoglobin is replaced by Hb S, acute sickledactylitis (hand-foot syndrome).

Pre-school: acute painful vaso-occlusion crises, affecting extremities.School children: painful crises affecting head, chest, abdomen, back, the site being typical for an any

individual patient. Episodes may be precipitated by intercurrent illness.Late changes:

o infarction of bone marrow or bone.

o splenic infarction between 6 and 60 months contributing to autosplenectomy.

o pulmonary infarcts (acute chest syndrome).

o stroke caused by cerebrovascular occlusion -/+ hemiplegia.

o ischaemic damage to myocardium, liver and kidneys, with progressive impairment of renal

function and concentrating ability.

Spleen changes: in young children the spleen is enlarged, with occasional acute splenic sequestration.

Altered splenic function increases the risk of serious infection particularly meningitis sepsis caused by

pneumococci and haemophilus influenzae (polysaccharide encapsulated organisms). As the child ages, auto

splenectomy reduces spleen size. Cardiomegaly is invariably present in older children (sickle-related


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cardiomyopathy), secondary haemosiderosis from increased iron absorption may damage liver, pancreas and

heart, and there may be gall stone formation. Puberty is frequently delayed, and chronic leg ulcers occur in

late adolescence.

79. Sickle cell trait is usually characterized by all of the following except:-

A. Red cell concentration of HbS is about 50%

B. Splenic infarction rarely occur except under extreme hypoxic condition

C. Reticulocyte count is usually elevatedD. In some individual hyposthnuria & hematuria may result from sickling in the renal medullary

capillariesE. The incidence in American black is 1 in 12

Ans:- C

80. In paroxysmal nocturnal haemoglobinuria:-

A. The defect is a congenital abnormality of RBC production by the bone marrow.

B. Hamoglobinuria is a characteristic finding.

C. Venous thrombosis is a recognised complication.

D. The diagnostic test is Hams test.

E. Transforms into acute leukaemia in approx. 5%. Ans:-BCDE

PNH is an acquired clonal defect of cell membranes that makes the cells more sensitive to lysis by

complement. This results in intravascular haemolysis and haemoglobinuria. Crises of haemolysis can be

triggered by infection, and chronic haemolysis may result in nephropathy. There is an increased risk of

venous thromboses (esp. in unusual sites such as saggital sinus or hepatic veins).The diagnostic test is

Hams test (acidified serum lysis). The disease may transform into acute leukaemia (in approx 5%).

81. The following conditions commonly require splenectomy in later life:

A. Sickle cell trait

B. Sickle cell diseaseC. Beta Thalassaemia

D. Glucose-6-phosphate dehydrogenase deficiency

E. Idiopathic thrombocytopenic purpura true

Ans:- C

Comments:Because of the risk of post-operative sepsis, splenectomy should be limited to specific indications. These

include:

Splenic rupture, anatomic defects.

Haemolytic anaemias, immune cytopenias. Metabolic storage diseases.

Secondary hypersplenism. Surgical indications (rare).

The major risk is infection, particularly in children less than 5 years. The risk of sepsis is slightly less insplenectomies done for trauma, red cell membrane defects, and immune cytopenias then when there is a pre-

existing immune deficiency such as Wiskott-Aldrich Syndrome or reticuloendothelial blockage such as

storage diseases or severe haemolytic anaemias.

82. The following is true of aplastic anaemia:-

A. Congenital aplastic anaemia is more common than the acquired form

B. Fanconi's anaemia is the commonest cause

C. Age related anaemia is prerequisite for the diagnosis to be confirmed

D. Erythropoietin is an effective treatment in the majority of cases

E. Allogeneic bone marrow transplantation is recognized treatment option

Ans:- E

83. Aplastic anaemia:


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A. Is defined as pancytopenia in the peripheral blood and blast cells in the bone marrow.

B. Has been associated with chloamphenicol eye drops.

C. Is preduced by hepatitis in 5-10% of cases

D. Is a feature of paroxysmal nocturnal haemoglobinuria

E. Can effectively be treated by growth factor injections

Ans:-CD

Aplastic anaemia is defined as the presence of pancytopenia in the peripheral blood and a hypocellularmarrow in which normal marrow is replaced by fat cells, abnormal cells are not found in either the

peripheral blood or bone marrow.Causes include: drugs, viral infections and paroxysmal nocturnal hemoglobinemia.

The latter condition is an acquired defect of the red cell membrane which is associated with intravascularhemolysis as the red cells are highly susceptible to complement lysis.

Many drugs have been implicated in aplastic anaemia but the evidence for many is slim.

Oral chloramphenicol not eye drops have been associated with aplastic anaemia, hepatitis, presumably of

viral origin is a precursor of aplastic anaemia in 5-10% of cases.

In the majority of cases no specific hepatitis virus is identified.

Definitive treatment is with immunosuppression or with allogenic stem cell transplantation in young patientswith HLA-matched doners.

Growth factors only have a role acutely to treat sever infection and are not effective as long term

treatment

84. These inborn errors have successfully been treated by bone marrow transplantation

A. Cystic fibrosisB. Hurler's syndrome.

C. Gaucher's syndrome.D. Fanconi's anaemia.

E. Chediak-Higashi syndrome.

Ans:-BCDE85. Regarding leukocytes:-

A. Eosinophils play role in the defense against parasites

B. Mast cell are derived from basophils

C. Once migrated into the tissue, neutrophil is called a phagocyte

D. Lymphocytes contain myeloperoxidase

E. Band cells are immature neutrophils

Ans:-ABE

86. 5 year old child is found to have neutrophil count of 51109 /L this may be due to :A. Acute liver failure.B. Corticosteroid therapy.

C. Brucellosis.D. Aplastic anaemia.

E. Hypersplenism.

Ans:-ABC

Granulocytes survive for only 612 hr in the circulation and, therefore, daily production of 2 104

granulocytes/L of blood is required to maintain a level of circulating granulocytes of 5 103/L. In

contrast, lymphocytes can exhibit lifetimes measured in months or years and require daily renewal of

lymphocyte progenitors at rates substantially lower than the other hematopoietic progenitors. The mean

WBC count at birth is high, followed by a rapid fall beginning at 12 hr until the end of the 1st week.

Thereafter, values are stable until 1 yr of age. A slow, steady decline in the WBC count continues

throughout childhood until reaching the adult value during adolescence. Leukopenia in adolescents andadults is defined as a total WBC count


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B. Increase susceptibility to viral infection.

C. Can be caused by corticosteroid.

D. May be cyclical.

E. May be complication of folate deficiency.

Ans:-DE

Viral Bacterial Fungal Protozoal

Respiratory syncytial virus

Dengue fever

Colorado tick fever

Mumps

Viral hepatitis

Infectious mononucleosis(EBV)

InfluenzaMeasles

RubellaRoseola

Varicella

Cytomegalovirus

Human immunodeficiency

virus

Sandfly fever

Pertussis

Typhoid fever

Paratyphoid fever

Tuberculosis

(disseminated)

Brucellosis

Tularemia

Gram-negative sepsisPsittacosis

Histoplasmosis

(disseminated)

Malaria

Leishmaniasis (kala-azar)

88. Atypical mononuclear cells are seen with :-A. Toxoplasmosis.

B. Herpes simplex.C. Rubella

D. Cytomegalovirus.E. Epstein-Barr virus.

Ans:-CDE

89. Neutrophil

A. Are present in normal tissues.

B. Are directed to sites of inflammation by IL18.

C. Phagocytes cells more efficiency when opsonised with antibody and complement as

compared with antibody alone.

D. Kill phagocytosed organisms by releasing granule contents

E. Deficiency increases susceptibility to viral infections.

Ans :-BCD

Neutrophils remain in the blood stream and only enter tissues if there is inflammation (unlike macrophages)

to which they are principally directed by IL18. Antibody and complement is a much more efficient opsoninthan antibody alone. Neutrophils release toxic oxygen compounds (respiratory burst),nitric oxide,

phospholipases and proteases from granules to kill phagocytosed organisms. Deficiency or dysfunction ofneutrophils results in recurrent bacterial and fungal infections

90. The following can cause an eosinophilia:-

A. Penicillin

B. HydralazineC. Polyarteritis nodosa

D. Pneumocystic carini

E. Atopic dermatitis.

Ans:- ABCDE


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Blood eosinophil numbers do not always reflect the extent of eosinophil involvement in disease-affected

tissues. The absolute eosinophil count, calculated as the white blood cell (WBC) count/L percent of

eosinophils, is usually 5,000 cells/l) eosinophilia, Patients with sustained bloodeosinophilia may develop organ damage, especially cardiac damage as found in the idiopathic

hypereosinophilic syndrome, and should be monitored for evidence of cardiac disease. Many cases ofmoderately severe eosinophilia often have no clear etiology.

ALLERGIC

DISORDERS

INFECTIOUS

DISEASES

MALIGNANT

DISORDERS

GASTROINTESTIN

AL DISORDERS

RHEUMATOLOGI

C DISEASE

I

Allergic

rhinitis

Tissue-Invasive

Helminth Infections

Trichinosis

Toxocariasis

Strongyloidosis

Ascariasis

Filariasis

Schistosomiasis

Echinococcosis

Brain tumorsInflammatory bowel

diseaseRheumatoid arthritis

Asthma Pneumocystis carinii

Hodgkin disease

and T-cell

lymphoma

Peritoneal dialysis Eosinophilic fascitis

Acute and

chronic

urticaria

ToxoplasmosisAcute myelogenous

leukemia

Eosinophilic

gastroenteritis

Pemphigoid AmebiasisMyeloproliferative

disordersMilk precipitin disease

Malaria Brain tumorsChronic active

hepatitis

Bronchopulmonary

aspergillosis

Hodgkin disease

and T-celllymphoma

CoccidioidomycosisAcute myelogenous

leukemia

ScabiesMyeloproliferativedisorders p

91. These statements are true:-

A. Methemoglobinemia is caused by oxidation of copper 2+ to copper 3+ form.

B. Methemoglobinemia may be due to HbC, abnormal hemoglobin.C. Methemoglobin reductase maintain Hb in it's reduced state.

D. NADH is the energy source used in the redox reaction.E. Production of NADH arise the consequence of conversion of 1, 3 diphosphoglycerate to

glyceraldehydes.Ans : -CD


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92. The following conditions present with purpura & normal platelet counts:-A. Osler-Weber-Rendu syndrome

B. Ehler Danlos syndrome

C. BernardSoulier syndrome.

D. Wiskott-Aldrich syndrome.

E. Scurvy.

Ans:-ABE

93. The following are causes of thrombocytopenia:

A. B12 deficiencyB. Chronic myeloid leukaemia.

C. Splenectomy.D. Bernard soulier syndrome

E. May-Hegglin anomaly.

Ans :-ABDE

B12 deficiency may be associated with a pancytopenia or with an isolated anaemia, thrombocytopenia or

leucopenia. Chronic myeloid leukaemia may be associated with a low, normal or high platelet count.

Bernard soulier syndrome and May Hegglin anomaly are congenital problems of platelet production and areboth associated with a thrombocytopenia. Splenectomy is associated with a thrombocytosis as well as

increased target cells and Howell Jolly bodies in the peripheral blood.

94. Thrombocytopenia occurs inApulmonary haemosiderosis.

B cavernous haemangioma.

C scurvy.

D infectious mononucleosis.

E chronic alcoholism.

Ans:- BDE

Comments:A - blood picture of iron deficiency +/-eosinophilia

C - perifollicular haemorrhage due to vascular weakness and impaired platelet function

cavernous haemangioma - platelet sequestration

chronic alcoholism - suppression of megakaryocytes

infectious mononucleosis - immune mediated

ThrombocytopeniaII. Signs

A. Bleeding disorders

B. Purpura orPetechiae

III. Causes: CongenitalA. Glanzmann's Thrombasthenia (autosomal recessive)

1. Platelet membrane deficiency Glycoprotein IIb, IIIa2. Defective binding of platelet Fibrinogen

3. Decreased platelet aggregation

B. Bernard-Soulier Disease (autosomal recessive)

1. Platelet membrane deficiency Glycoprotein Ib

2. Coagulation Factor X and Factor V deficiency

3. Large platelets and decreased platelet aggregation

C. Storage Pool Disease

1. Dense granule and/or alpha granule deficiency

2. Defective platelet release of ADP and Serotonin

IV. Causes: AcquiredA. Uremia

B. Chronic Liver Disease


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C. Medications1. Aspirin

2. Furosemide (Lasix)

3. Nitrofurantoin (Furadantin)

4. Heparin5. Sympathetic blockers

6. Clofibrate (Atromid-S)7. NSAIDs

95. The following are recognised causes of drug-induced immune thrombocytopenia:-

A. ChloramphenicolB. Quinine

C. Penicillamine

D. Rifampicin

E. Heparin

Ans:-BDE

All the above drugs can cause thrombocytopenia. However chloramphenicol, penicillamine andphenylbutazone cause this by bone marrow suppression. The others produce an antibody-drug-plasma

protein complex which deposits on the platelet surface leading to either lysis by complement or removal by

reticuloendothelial cells. Patients present with acute purpura and platelets often less than 10x10 9/l. Heparin

also causes thrombocytopenia through platelet aggregation.

96. Hemophilia A:-A. Is the commonest inherited bleeding disorder.

B. Inheritance is autosomal dominant with variable penetrance.

C. Severity is determined by level of factor VIIIc

D. Is treated with factor VIII given by IM injection.

E. Is no longer treated with prophylactic factor VIII because of HIV risk.Ans:-AC

Recombinant factor VIII is not widely available.

97. The following statement concerning hereditary bleeding disorder are correct :-

A. HCV is common cause of liver disease in adults with sever hemophilia treated before the1990.

B. Antenatal diagnosis of hemophilia A is possible.C. Von-Willebrand disease is inherited as an autosomal recessive manner in the majority of

patient.D. Children with hemophilia have normal prothrombin time.

E. In hemophilia A , spontaneous bleeding into the joint occur when the factor VIII

concentration is reduced to 20% of normal.

Ans:-ABD

98. The following condition are caused by an abnormality of Hb synthesis :-

A. Beta thalassemiaB. Congenital spherocytosis

C. Pyruvate kinase deficiencyD. Hemophilia A

E. Sickle cell disease.Ans:-AE

99. Von Willebrand disease (vWD):-A. Is caused by pualitative or quantitative deficiency of von Willebrand factor

B. The resulting bleeding disorder in vWD is characterized by petechiae

C. The platelet count is normal

D. The ristocetin co-factor activity will be decreased


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E. Is inherited in an X-linked recessive pattern

Ans:-ACD

100. Hemophilia:-A. Doesn't cause coagulation disturbance in the newborn.

B. Lack positive family history in 1/3 of the affected boys.

C. Cause profound bleeding time.

D. Cause prolonged APTT ( test of intrinsic coagulation pathway).

E. Result from the synthesis of biologically inactive factor VIII (AHG).

Ans:-BDE

101. Which of the following is true of Factor VIII antihaemophilic globulin?

A. released mainly by megakaryocytes

B. mediates the endothelial platelet aggregation

C. is an essential co-factor in the activities of Factor X to Factor Xa

D. has a half life of 36 hours

E. deficiency is a major source of bleeding in Von Willebrand's disease

Ans:-BCDE

Comments:factor VIII antihaemophilic globulin / factor = Von Willibrand's factor It is the plasma carrier for factor VIII

a-mainly from endothelium but also from megakaryocytesb-it links platelet membrane receptors to vascular subendothelium

c-in association with calcium / activated factor IXd-stored blood at 4C -> activity falls to 10% in first 3 days (Dr Shu Ho)

102. Regarding coagulation:-

A. All clotting factor are synthesized in the liver

B. Factor VIII is synthesized by hepatocytes and by endothelial cells

C. FactorCIII deficiency doesn't prolong the INR nor APTT

D. An isolated factor VII deficiency elevates the APTT onlyE. Isolated factorII deficiency result in prolonged APTT, thrombin time( TT) and INR

Ans:-ABCE

Because factor XIII is responsible for the cross linking of fibrin to stabilize the fibrin clot, symptoms ofdelayed hemorrhage are secondary to instability of the clot. Typically, patients have trauma 1 day and then

have a bruise or hematoma the next day. Clinical symptoms include mild bruising, delayed separation of theumbilical stump beyond 4 wk, poor wound healing, and recurrent spontaneous abortions in women

103. The following are component of intrinsic coagulation:-

A. Prothrombine

B. Factore CP

C. Factore IC.

D. Fibrinogen

E. Factore VII

Ans:-BC

104. In neonatal alloimmune thrombocytopenia:-A. The most commonly occurring human platelet antigene is HPA-1a

B. The father need to be HPA negative and the mother positive for the same HPA for NAIT todevelop

C. The first born child of genetically predisposed parents is commonly involved.D. The thrombocytopenia become evident after approximetly 5 days of life.

E. The treatment includes antenatal periumbilical transfusion of maternal donated platelet.Ans:-ACE

105. Neonatal thrombocytopenia is recognized feature of :-A. SLE

B. Maternal ingestion of thiazid diuretic.


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C. Maternal IgM antibodies.

D. In utero infection with Toxoplasmosis.

E. Shortened forearm.

Ans ;-ADE

106. Thrombocytopenia has recognized association with :-A. HSP

B. Septicemia.C. Rubella.

D. Von-Willebrand disease.E. Portal hypertension.

Ans:- BCE

107. Idiopathic Thrombocytopenic Purpura (ITP) in Childhood:

A. Is usually self limiting.

B. Always requires treatment if the platelet count is less than 20 x 109/L.

C. Is associated with cerebral haemorrhage in less that 1% of cases.

D. Can be successfully treated with steroids.

E. Can be successfully treated with intravenous immunoglobulins. Ans:- ACDE

Comments:Acute idiopathic thrombocytopenic purpura is the most common of the thrombocytopenic purpuras of

childhood. It is associated with petechiae, mucocutaneous bleeding, and occasionally haemorrhage into the

tissues. There is a profound deficiency of circulating platelets despite adequate numbers of

megakaryocytes in the marrow. In 70% of cases there is an antecedent disease or viral infection. ITP has anexcellent prognosis even when no specific therapy is given. Within 3 months, 75% of patients recover

completely, mostly within 2 months. Spontaneous haemorrhage and intracranial bleeding (20

109/L,)

110. Idiopathic thrombocytopenia purpura:A. Result from decreased platelet production.

B. Classically present with acute blood loss.


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C. Require urgent treatment to prevent intracranial hemorrhage.

D. Should be confirmed b

mcq of hematology

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