Hematological Disorders

First semester

Nur 221

Hematological Disorders

• The hematological system consists of the blood and the sites where blood is produced

• The blood make 7-10% of body wt and amount to 5-6 L of volume.

• Blood composed of plasma and cells

• Blood is in the fluid status, and 55% is plasma

• Plasma contain proteins like albumin, globulin, fibrinogen and factors necessary for clotting, & electrolyte.

• Cells of the blood are (WBC's, RBC's, platelets), make up 40-45% of blood volume.

• Hematopeiesis: is the process through which the blood is generated from the bone marrow (vertebrae, pelvis, ribs, sternum in adults).

• The blood carry O2 absorbed from the lungs and nutrients absorbed from the GI tract to the body cells for cellular metabolism. It carries hormones, antibodies and other substances to their sites of action.

• Blood carry waste products by cellular metabolism to the lung, skin, liver and kidney to be eliminated from the body.

• RBC's: biconcave disk that resembles a soft ball compressed between two fingers. They consists primarily from Hb.

• WBC's: protect the body from the invasion of bacteria.

• Platelets: helps in controlling of bleeding. They get activated when vascular injury happen: adhere to the site and to each others forming a plug that stop bleeding: substances released from the platelets activates coagulation factors in the blood plasma to formulate a stable clot (fibrin). Normal life span of 7-10 days.

• Plasma: the liquid portion of the blood is called plasma. Consists of 90% water, and proteins (albumin and globulin) and clotting factors (fibrinogen), electrolytes

Assessment of hematologic system

• Hematologic studies:

- CBC ( WBC’s, and its differential, RBC’s and there indices, Hb, HCT, Plat

- Peripheral blood smear

• Bone marrow aspiration & biopsy

• Bone marrow aspiration

Common sites for aspiration are the sternum and the iliac crest in the hip boneMost common complications of the procedure are infection and bleeding

Bone marrow aspirationAfter the biopsy

• You will lie down for 10 to 15 minutes after the biopsy so the site can be checked for bleeding. Your bandage can be taken off after 1 hour. If you had a sedative, you will need someone to drive you home after the biopsy.

• You may feel sore at the biopsy site for several days. Ice packs to the site, walking, and pain medicine, such as acetaminophen (Tylenol), can be used to help you feel better.

Call your doctor immediately if you have:

• More tenderness, pain, redness, or swelling at the biopsy site.

• A fever.

• Bleeding or drainage, such as pus, from the biopsy site. If you are bleeding, put pressure on the site and call your doctor.

Anemia

• Anemia: a condition in which the Hb concentrations is less than normal, reflects the presence of fewer than normal RBC's within the circulation thus O2 delivered to body tissue is diminished.

• It is not a disease per se it is a sign for underlying disorder

• Occur in all age group, prevalent among elderly

Anemia depends on:

• The marrows’ ability to respond to the decreased RBC's.

• The degree to which young RBC's proliferate in the bone marrow and the manner in which they mature

• The presence or absence of end products of RBC's destruction within the circulation (increase billirubin, and haptoglobin level)

• Anemia can be classified according to the physiological defect: (Classification of Anemia: table 33-2)

• Defect in their production (Hypoproliferative anemia):RBC # is < normal due to bone marrow defect due to

medications or chemicals or lack of factors important for RBC's production such as (iron, folic acid, erythropoietin)

• Destruction (hemolytic): premature destruction of RBC's. Results in tissue hypoxia; the released Hb is resulted in ↑billirubin concentration. RBC's destruction can results from abnormality in the RBC's such as Sickle cell anemia, glucose-6-phosphate dehydrogenase deficiency, or within the plasma (immune hemolytic anemia) or direct injury to erythrocytes.

• Loss (bleeding)

Causes of hemolytic anemia (Chart 33-1)

• Inherited hemolytic anemia: • abnormal hemoglobin (thalassemia, sickle cell anemia), • RBC's membrane abnormalities, • enzyme deficiency (G-6-PD)

• Acquired hemolytic anemia: – Antibody related (transfusion reaction), Autoimmune

hemolytic anemia – Not-antibody related: RBC's membrane defects, liver

disease, uremia, mechanical heart valve, bacterial parasitic infection, toxins

Clinical manifestation

Factors influence development of anemia associated symptoms:

- depends on the speed with which anemia developed

- duration of anemia- metabolic requirements of the individual- other concurrent disorder (cardiovascular).

• Patients who has anemia for a long time (Hb 9-11g/dl), develop no symptoms with slight tachycardia on exertion and fatigue.

• The more rapid the more severe the symptoms

• very active persons develop symptoms more than sedentary persons; hypothyroidism with less O2 needs may not develop symptoms.

• Pt with cardiac, vascular and pulmonary disease develop more pronounced symptoms of anemia (chest pain, dyspnea, muscle pain and cramping)

Diagnostic findings

• Hb, Hct, reticulocyte count, RBC indices (such as mean corpuscular volume (MCV).

• Iron studies: serum iron level, Transferrin or total- iron binding capacity (TIBC), serum vit B12, folate level, serum ferritin

• Bone marrow aspiration• Other Dx is useful to determine if the anemia is

separated problem or as a result of other disease such as malignancies, sources of blood loss (such as ulcer in the GI tract)

• Haptoglobin and erythropoietin level

• Complications:Heart failure, confusion or delirium, paresthesias, angina symptoms.

• Medical Management: is aimed to correct and control the cause of anemia, if severe PRBC's can given through blood transfusion

Nursing processAssessment:• Pt history (Hx) and examination, • fatigue, malaise, pallor of skin and mucus membrane

(conjunctiva, oral mucosa)• Family Hx (some anemia are inherited)• Jaundice (hemolytic, Megaloblastic anemia) • Tongue (smooth red in iron deficiency anemia), beefy red & sore

in Megaloblastic anemia), angular cheilosis (in both types) • Medication Hx (medication can suppress bone marrow). • Nutritional assessment: deficiency in iron, folate, B12, • Cardiac assessment: Heart try to compensate harder & faster in

anemia (tachycardia, palpitations, dyspnea, dizziness, Orthopnea, heart failure may develop [cardiomegaly or hepatomegaly and peripheral edema])

• Assess GI for blood loss (coffee ground vomitus, melena. • Neurological assessment: peripheral numbness, ataxia, poor

coordination, confusion. • Lab test

Nursing diagnosis

• Fatigue R\t ↓Hb

• Activity intolerance related to weakness, fatigue

• Imbalanced nutrition, less than body requirements, related to inadequate intake of essential nutrients

• Altered tissue perfusion related to inadequate blood volume or hematocrit

• Noncompliance with prescribed therapy

• Collaborative problem:

- HF

- Angina

- Parasthesia

- confusion

Planning:

• Managing fatigue: balance between activity and rest

• Maintaining adequate nutrition

• Maintaining adequate perfusion

• Promoting compliance with prescribed therapy

Intervention: • For managing fatigue:- Assess pt to prioritize activity - Establish balance between rest and activity• For maintaining adequate nutrition:- Encourage healthy diet - Avoid alcohol- Nutritional supplement may be advised • For maintaining adequate perfusion:- Provide O2- Monitor V\S & pulse oximeter- Intravenous fluid or blood may be administered

• To promote compliance with therapy:

- Explain to the pt the purpose of medication, how to take it and about S\E

- assist pt in adjust the plan into every day activity

• Monitoring and managing complications:

- Assess pt for S&S of heart failure

- Daily wt

- A neurological assessment

Hypoproliferative anemias• Iron deficiency anemia, vit B12 deficiency

(megaloplastic) anemia & aplastic anemia.

• Iron deficiency anemia ( IDA, Hypochromic-Microcytic): results when dietary intake of iron inadequate for Hb synthesis; the body store only one fourth or one third of its iron

• most common type of anemia in all age group, more than 500 million people develop it

• Most susceptible very young, those on poor diet, and women in their reproductive age children and adolescent

• The most common cause of IDA in adult men and postmenopausal women is bleeding from (peptic ulcers, gastritis, GI tumor, inflammatory bowel disease)

• In pregnant women due to not taking iron supplement;

• In premenapausal: heavy menstrual bleeding

• Chronic alcoholism

• Malabsorption (as in gastrectomy, or defect in duodenum mucosa)

Symptoms

• In early course no symptoms• When it become chronic general manifestation of

anemia occur• Specific symptoms:

- Pallor and glossitis (smooth sore tongue) most common

• brittle and ridged nails, angular cheilosis (ulceration at the corner oft the mouth), headache, burning sensation at the tongue

• Abnormal craving for food (pica) as for ice and clay

Diagnostic findings

• Bone marrow aspiration is the most definitive method used (iron)

• Other finding: S. ferritin, MCV, MCH, MCHC, HB,

RBC’s, S. iron, transferrin (TIBC)

- S. ferritin is the most sensitive test

Medical management • Investigate causes of iron deficiency anemia such as:

- intestinal cancer, or of uterine fibroid tumors, ulceration, (using endoscopy, colonoscopy & stool specimen for occult blood, X-ray to the GIT).

• Administer oral iron supplements-ferrous sulfate, ferrous gluconate, & ferrous fumarate.

• It may take longer time for iron store to be replenish so patient must be advised to take iron for as long as 6-12 months.

• If iron supple orally is poorly tolerated, & pt need large amounts. Then iron dextran should be administer through IV or IM injection. Check for allergic reaction with small dose for the first 30 minute then administer the rest of the iron.

Nursing management

• Nutritional counseling: Food source high in iron (beef or calf’s liver, chicken liver). Other meats, beans (blacks, garbanzo), leafy green vegetables, raisins, and molasses.

• Take all food rich in iron. • Take this type of food with Vit C • If pt take medication advise him/her to take it before meal

(1hr); Iron best absorbed on an empty stomach. If it caused some distress take it with meal. Do not take antacids with it sense it decrease its absorption

• Iron supple may have adverse effect on GI: nausea, vomiting, cramping.

• Instruct pt to take fiber diet…. Some supp may be taken with stool softeners.

• Give pt instruction how to prevent GI distress • If liquids form is taken : it may cause stain so take it with

straw and rinse the teeth after taking it.

• Instruct pt that it may cause dark stool or dark green.

• IM may cause some local pain or stain of skin; avoid extra rubbing to the place and use Z-track technique deeply in gluteus maximums muscle

Aplastic anemia• Rare disease, also called hypoplasia,

pancytopenic anemia

• caused by damage or decrease in marrow stem cells due to damage to the microenvironment of the marrow or replacement of the marrow with fat which results in markedly marrow aplasia (↓hematopoiesis).

• Thrombocytopenia & neutropenia may be also seen with it.

pathophysiology

• Idiopathic (most cases 70%),• Congenital (due to chromosomal abnormality)

• Acquired from: chemical (as benzene or its derivatives), medications (chemotherapy, anti seizures), infection (hepatitis), toxins like pesticides

• triggered by pregnancy & infection • One hypothesis: is that body T- cell mediate

inappropriate attack against the marrow cause marrow aplasia

• C\M:

- Pallor

- Fatigue

- Dyspnea

- Infection (chest, lymphadenopathy)

- Bleeding (petichiae, epixtasis)

Assessment and Dx

• Review medication history: since toxins and some medication may be the cause

• Bone marrow aspirate may show hypoplastic or aplastic (very few to no cells), fat marrow.

HB & HCT, Normal RBC's indices, WBC’s, RBC’s, platelet

Medical management • Bone marrow transplantation (BMT) if there is

a donor for young people.

• Peripheral blood stem cell transplantation

• Immunosuppressive therapy ( cyclosporine, antithymocyte globulin (ATG) to prevent the lymphocyte from destroying the stem cells for older pt.

• Transfusion of RBC's and platelets as necessary.

• Nurses should monitor infection and bleeding which may cause death in aplastic anemia

Megaloplastic Anemia

• ↓ of vit B12 or folic acid, bone marrow or peripheral blood changes occur because both vits. are essential for DNA synthesis.

• Results in production of large RBC's called megaloplastic RBC

• Bone marrow analysis shows hyperplasia: abnormal ↑ in the number of cells and the precursor erythroid and myeloid cells are large and bizarre in appearance. RBC’s can be destroyed at the marrow or they leave the marrow mature but very few in number, pancytopenia (in all myeloid derived cells) may be presents.

• Hb can be 4 to 5 g/dl. WBC count 2000-3000/mm³ and the platelets less than 50,000/mm³. RBC's, platelets are abnormal large and RBCs in bizarre shape, MCV is high > 110μm³.

Size and shape of RBCs: ( large and bizarre appearance )

Pathophysiology:

1-Folic acid deficiency

• Folate storage in the body are small & deplete quickly in 4 months

• Folic acid is found in uncooked green vegetables and liver

• This anemia can be found pregnant women & in people with malabsorption diseases.

2-Vit B12 deficiency:

• The body has large stores of B12 so anemia may not be apparent in years

• Causes of B12 deficiency:

- In strict vegetarians (inadequate intake)

- Decrease absorption from GIT as in (Crohns’ disease),

- Abnormality in the gastric mucosa or stomach wall atrophy (fails to excrete intrinsic factors and bind with B12 to be absorbed) this called pernicious anemia; or in gastrectomy

• Clinical manifestations: in both types of anemia are similar; in B12 anemia there are some neurological symptoms. Therefore, serum level of both Vit B12 and folate must be measured.

• Manifestations of anemia appear very slowly (weakness, fatigue, lethargy, listlessness).

• Very pale, smooth sore tongue, mild diarrhea,

• Confusion, numbness in the extremities, loss of position sensation (proprioception), difficulty maintaining their balance (ataxia) due to spine damage.

• May last several year if not treated and eventually death may happened due heart failure because of anemia.

Assessment and diagnostic findings• Red cell folate is the sensitive test to determine folic acid

deficiency anemia • Schilling test for vit B12 deficiency: small radioactive oral B12

dose is administered and followed in few hours with non radioactive parenteral dose of B12. if the oral B12 is absorbed; then more than 8% of it will be excreted in the urine; if no radioactive is present in the urine then the vit b12 stays in the GI; the cause is GI Malabsorption of vit b12.

• Therefore, If radioactive present in the urine it means the cause of anemia is not pernicious anemia or ileal disease.

• Adding intrinsic factor to radioactive oral B12 : if detected in urine …. pernicious anemia

• Methyl- mylonic acid increase in B12 deficiency (common)• The intrinsic factor antibody test (easier) : +ve indicate the

presence of antibodies that bind with B12 and prevent it from binding to receptors in the ileum and thus preventing its absorption

Medical management:• Folate deficiency can be treated with folic acid in the diet

and administer 1 mg of it daily orally or IM (if not tolerated orally; & for people with Malabsorption problem)

• Vit B12 treated with B12 replacement; with orally vit or fortified Soya milk.

Nursing management: - assess signs and symptoms - Pay attention to ambulation, safety, coordination, and asses

patient gait and stability and the need for assistive devise.

- Since mouth and tongue sores are present encourage pt to have small, soft frequent meals.

- Teaching about follow up and screening and severity of the disease

Hemolytic anemia

• Short life span for the RBC's, ↓O2 cause hypoxia, 80 % of the heme is converted to bilirubin conjugate in the liver and excreted in the bile

• All type of hemolytic anemia share same lab feature (reticulocyte, indirect bilirubin, haptoglobin)

• Haptoglobin (is a blood protein made in liver, bind to free Hb)

Sickle cell anemia• severe hemolytic anemia results from inheritance of

sickle hemoglobin gene.• RBC's containing HbS loss its round, biconcave disk

shape & becomes deformed, rigid and sickle shaped. • It results in ischemia and infarction due to adhesion

into endothelium, patient may have pain, swelling and edema, or may adhere to each others and make occlusion of arterioles.

• Can be in different shapes: sickle cell hemoglobin C (SC) less sever; Sickle cell hemoglobin D (SD) and sickle cell beta-thalassemia.

• Sickle cell carrier: less than 50% of Hb is HBS

RBC's becomes deformed, rigid and sickle shaped

• Sickle cell crisis triggered by:

- Cold due to vasoconstriction

- Increase blood viscosity

- High altitude

- (infection, cold, dehydration, and emotional stress)

Clinical manifestations: • Always anemic Hb 7-10 g/dl• Jaundice, • enlarge face bone & skull (because bone marrow

try to compensate the bones). • Chronic: tachycardia, murmur, cardiomegaly in

adults dysrhythmias & heart failure. Hypoxic damage of vital organs.

• Complications: infection, stroke, renal failure, impotence, heart failure, CVA, skin ulcers, eye: retinal detachment & blindness, pulmonary hypertension, acute chest syndrome.

Sickle cell crisis three types:• Very painful crisis: due to hypoxia and necrosis from

inadequate bld flow to organ• Aplastic crisis: from infection, Hb falls rapidly and bone

marrow cannot compensate (absence of reticulocyte) • Sequestration crisis: when organ pool the sickle cells; in

children spleen infarction by age 10y and no longer functioning; in adults liver and lungs.

• The SCA can diagnosed in childhood; infants become anemic and have crisis at year 1 or 2 then they may die at the 1 year of life. Can manage to 42 years old and can cure in the 30s of age…depends on the severity of it

Diagnosis of SCA

• Pt with sickle cell trait have normal lab test

• With disease he will have decrease in HCT, sickle cell in blood smear

• The SCA diagnosis confirm with Hb electrophoresis

Medical management

• BMT : few people may have due to incompatible donors or the severe organs damage already

• Pharmacological therapy: hydroxyurea (hydrea): a chemotherapy (increase HbF level); decrease the permanent formation of sickled cells. They decrease the painful crisis, have lower incidence of acute chest syndrome and less need for blood

• Side effects: chronic suppression of WBC formation, and later development of malignancy, patients may develop liver toxicity at low dose

• Arginine: increase nitric oxide (N.O) availability so decrease pulmonary artery pressure

• Transfusion therapy: essential to prevent complications of SCA. 90% reduction of stroke in children.

• Risks include: iron overload, poor venous access; infection (Hepatitis); ↑ concentration of Hb. Need very close observation specially for hemolytic reaction because with repeated transfusion patient develop auto antibodies which made the cross matching difficult, ↑Bld viscosity

• Daily folic acid replacement needed to ↑ erythropiosis• Antibiotic to treat infection the major cause of death; to

decrease chest infection: antibiotic and spirometer; bronchoscopy; corticosteroid given

• Supportive therapy: pain management is essential; pain can last hours to days and vary from one to another. Aspirin, NSAID, and morphine can be used.

• Adequate hydration and O2 therapy

Nursing process for patient with SCA

Assessment: • Assess factor that precipitate crisis (infection, cold,

dehydration, and emotional stress)• Assess pain (full Ch.Ch). • Assess of body system for signs of hypoxia• assess resp system, neurological system, cardiac

system • signs of infections and Lab test for HB and HCT • previous medical history or bld transfusion history• Assess for abd pain and tenderness (due to splenic

infarction)

Possible nursing Dx

• Acute pain related to tissue hypoxia due to agglutination of sickle cells within the body vessels

• Risk for infection

• Risk for powerlessness related to illness induced helplessness

• Deficient knowledge regarding sickle cells prevention

• Activetity intolerance R\T hypoxia

• Planning:

- Relief pain

- Prevent infection

- Enhance sense of power

- Increase knowledge

- Decrease incidence of crisis

Nursing interventions are aim to

• Manage pain

• Prevent and treat infection: if oral complete the entire course

• Promoting coping skills: positive self esteem trust.

• Increase knowledge: maintain adequate hydration to prevent the severity and occurrence of the attack

• Teach pt to avoid cold environment and stressful situation

Blood & Blood components Therapy

Blood & Blood components Therapy

• Blood unit is 450 ml+50 ml anticoagulant; can be separated into PRBC's, platelets, plasma (can be separated into albumin, immune globulin, factor VIII, Factor IX).

• PRBC's should be stored at 4 C and stored safely for up to 42 days

• Platelets should be stored at room temp and last only for 5days

• plasma should be immediately frozen to maintain activity of clotting factors and last for 1 year, if kept frozen

Blood donation: • To protect both the recipient and the donor you should

review the following types of history in donor:• Viral hepatitis,• infusion or transfusion of any blood derivative• untreated syphilis or malaria, • drug abuse, • HIV, • skin infection, • asthma, pregnancy, tooth extraction within 72 hours,

exposure to infectious disease, recent immunization, tattoo, cancer & blood donation (within the past 56 days)

• Body weight should exceed 50 Kg, > 17 yrs old not qualified, stable VS, Hb 12.5 g/dl women and men 13.5 g/dl

Administering blood and blood components: • Review the order • Review institutional policy. • consent form • Explain the procedure• Take VS • Instruct pt about signs of reaction to blood components

(itching, swelling, SOB, fever, chills, flushing).• Use 2o-gauge needle or larger for placement in large vein. • Wear gloves • Double check on the ABO and Rh type, • Check pt name and label.

The procedure: • Obtain the blood from the blood bank after starting the IV

line.

• Double check on Dr.order , pt name & labels • Initiate the transfusion within 30 min• In the first 15 min start slowly no faster than 5 mL/min if

no signs of adverse effect increase the rate• Infusion time not more than 4 hours because of

increasing bacterial proliferation

• Check closely for 15-30 min any signs for reaction and vital signs,

• Observe signs of adverse reactions: circulatory overload, sepsis, febrile reaction, allergic reaction, acute hemolytic reaction

• Change blood tubing every 2 units transfused

After the procedure:

• Obtain vital signs and compare baseline measurement

• Dispose materials carefully

• Document the procedure (pt assessment finding and tolerance)

• Monitor pt response to and effectiveness of the procedure

Alert

• Never add medication to blood or blood products.

• Blood should be warmed

• If blood is too thick to run freely you can add normal saline to the unit.

Complications• Febrile reaction:- Caused by antibodies to donor leukocytes that

remain in the unit of blood - S&S: chills, fever (2hr’s after transfusion), muscle

stiffness• hemolytic reaction:- dangerous, lifethretining- Occur when donor bld incompatible with recipient

blood- S&S: fever, chills, nausea, chest tightness and

dyspnea, hypotension, hemoglobinuria- Rx: D\C transfusion, obtain urine and blood sample.

• Allergic reaction:- Due to sensitivity from plasma proteins within blood

component- S&S: generalized itching, flushing, in sever reaction they

may develop laryngeal edema, bronchospasm- Rx: antihistamine if symptoms resolve transfusion

resumed, in sever reaction epinephrine & corticosteroid given.

• Circulatory overload: - Occur from infusing large amount too quickly, mainly in

pt with circulatory problem as (HF)- In pt with circulatory overload diuretics are administered

after transfusion or between units- S&S: dyspnea, Orthopnea, tachycardia, jugular vein

distended, crackles, hypertension- Rx: place pt in upright position with leg in dependant

position, D\C transfusion, notify physician, O2 therapy and morphine may be needed

Nursing intervention for transfusion reaction

• Stop transfusion• Maintain IV line with NS• Assess pt carefully• Obtain V\S• Notify Dr• Send bld bag and tubing for bld bank for culture• In hemolytic reaction, obtain bld specimen and

urine specimen for hemoglobinuria • Document the reaction