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Hematology

Blood

Blood is composed of:PlasmaRBCWBCWBCPlatelets

Plasma

Plasma consists of:90% water.

10 % solutes: albumin, electrolytes and proteins.y p

Proteins consist of clotting factors, globulins, circulating antibodies and fibrinogen.

Red Blood Cells

RBC’s travel through the body delivering oxygen and removing waste.RBC’s are red because they contain a protein chemical called hemoglobin which is brightchemical called hemoglobin which is bright red in color.Hemoglobin contains iron, making it an excellent vehicle for transporting oxygen and carbon dioxide.

RBC’s

Average life cycle is 120 days.The bones are continually producing

llnew cells.

White Blood Cells

The battling blood cells.The white blood cells are continually on the look out for signs of disease.When a germ appears the WBC will:When a germ appears the WBC will:

Produce protective antibodies.Surround it and devour the bacteria.

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WBC’s

WBC life span is from a few days to a few weeks.WBC’s will increase when fighting infection.

Platelets

Platelets are irregularly-shaped, colorless bodies that are present in blood.Th i ti k f l tTheir sticky surface lets them form clots to stop bleeding.

Blood Values

CBC with differential and platelet count.Hgb:

Normal levels are 11 to 16 g / dlPanic levels are:

Less than 5 g / dlMore than 20 g / dl

Hematocrit

Normal hematocrit levels are 35 to 44%.Panic levels:

Hmct less than 15 %Hmct greater than 60%

Hemoglobin and Hematocrit

Can be used as a simple blood test to screen for anemia.The CBC with differential would be used to help diagnose a specific disorderhelp diagnose a specific disorder.A bone marrow aspiration would be the most conclusive in determining cause of anemia –aplastic / leukemia.

Bone Marrow

Bone marrow is the spongy substance found in the center of the bones.

• It manufactures bone marrow stem cells, which in turn produce blood cellswhich in turn produce blood cells.• Red blood cells – carry oxygen to tissue• Platelets – help blood to clot • White blood cells – fight infection

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Bone Marrow Transplant

Donor is placed under anesthesia.Marrow is aspirated out of the iliac crest.Marrow is filtered and treated to remove bits of bone and other unwanted cells and debris, ,transferred to a blood bag, and is infused into the patient’s blood just like at transfusion.

Bone Marrow Aspiration

Treatment Modalities

Transfusion:Packed red blood cells – anemiaPlatelets – platelet dysfunctionFresh frozen plasma – coagulation factorsFresh frozen plasma coagulation factors

Blood Transfusions

3 types of transfusion reactionsHemolyticAllergicFebrileFebrile

Hemolytic Reaction

Refers to an immune response against transfused blood cells.Antigens, on the surface of red blood cells, are recognized as “foreign proteins” and canare recognized as foreign proteins and can stimulate B lymphocytes to produce antibodies to the red blood cell antigens.

Hemolytic reaction

Flank painFeverChillsBl d iBloody urineRashLow blood pressureDizziness / fainting

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Management

Stop the blood transfusion.Start normal saline infusion.Take vital signs with blood pressureObtain blood sample and urine specimen.p pReturn blood to blood bank.Document

Febrile Reaction

Often occurs after multiple blood transfusions.Symptoms:fever, chills, and diaphoresis.Interventions:Interventions:

Slow transfusion and administer antipyretic.Administer antipyretic prior to administration.

Allergic Reaction

Symptoms: rash, urticaria, respiratory distress, or anaphylaxis.I t tiInterventions:

administer antihistamine before transfusionPhysician may order washed rbc’s

Hematologic Conditions

Alteration in Hematologic Status

Disorders of hemostasis or clotting factorsStructural or quantitative abnormalities in the hemoglobin.AnemiasAnemiasAplastic Anemia

Genetic Implications

The following have a genetic link: implications for genetic screening and fetal diagnosis

Sickle cell anemiaThalassemiaThalassemiaHemophilia

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Bleeding Disorders

Three types Hemophilia: males onlyType A most common – factor VIII deficiencyType B - lack of factor IX (Christmas Disease)Type C – lack of factor XIType C lack of factor XI

Von Willebrand Disease – 1% of population – men or women – prolonged bleeding time

Hemophilia Type A

Hemophilia type A is the deficiency of clotting factor VIII.

A serious blood disorderAffects 1 in 10,000 males in the USAffects 1 in 10,000 males in the USAutoimmune disorder with lowered level of clotting factorAll races and socio economic groups affected equally

Hemophilia

Hemophilia is a sex-linked hereditary bleeding disorderTransmitted on the X chromosomeFemale is the carrierFemale is the carrierWomen do not suffer from the disease itself

Historical Perspective

First recorded case in Talmud Jewish text by an Arab physician – documentation of two brothers with bleeding after circumcision.Queen Victoria is carrier and spread theQueen Victoria is carrier and spread the disease through the male English royalty.

Goals of Care

Goals of care:Provide factor VIII (IX) to aid blood in clotting.To decrease transmission of infectious agents in blood products; hepatitis & AIDS.p pFuture: gene therapy to increase production of clotting factor.

Symptoms

Circumcision may produce prolonged bleeding.As child matures and becomes more active the incidence of bleeding due to traumathe incidence of bleeding due to trauma increases

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Symptoms

May be mild, moderate or severeBleeding into joint spaces, hemarthrosisMost dangerous bleed would be intracranial.

Diagnosis

Presenting symptomsProlonged activated aPTT and decreased levels of factor VIII or IX.Genetic testing to identify carriersGenetic testing to identify carriers

Treatment

Products used to treat hemophilia are:Fresh frozen plasma and cryoprecipitate which are from single blood donors and require special freezing.Second generation of factor VIII are made with animal or human proteins.

Teaching

Avoid aspirin which prolongs bleeding time in people with normal levels of factor VIII.A fresh bleeding episode can start if the clot becomes dislodged.Natural reactions in the body cause the clot that is no longer needed to “break down. This process occurs 5 days after the initial clot is formed.

Family Education

Medic-Alert braceletInjury prevention appropriate for ageSigns and symptoms of internal bleeding or hemarthrosishemarthrosisDental checkupsMedication administration

Long Term Complications

20% develop neutralizing antibodies that make replacement products less effective.Gene therapy providing continuous production of the deficient clotting factorproduction of the deficient clotting factor could be the next major advance in hemophilia treatment.

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Disseminated Intravascular Coagulation or DIC

DIC is an acquired coagulopathy that is characterized by both thrombosis and hemorrhagehemorrhage.DIC is not a primary disorder but occurs as a result of a variety of alterations in health.

Assessment

The most obvious clinical feature of DIC is bleeding.Renal involvement = hematuria, oliguria, and anuria.Pulmonary involvement = hemoptysis, tachypnea, dyspnea and chest pain.Cutaneous involvement = petechiae ecchymosisCutaneous involvement = petechiae, ecchymosis, jaundice, acrocyanosis and gangrene.

Management of DIC

Treatment of the precipitating disorder.Supportive care with administration of platelet concentration and fresh frozen plasma and coagulation factors.Administration of heparin (controversial in children).Heparin potentates anti-thrombin III which inhibits thrombin and further development of thrombosis.

Prognosis

Depends on the underlying disorder and the severity of the DIC.

ITP

Idiopathic thrombocytopenic purpuraIdiopathic = cause is unknownThrombocytopenic = blood does not have enough plateletspPurpura = excessive bleeding / bruising

Immune Thrombocytopenic Purpura

Antibodies destroy plateletsAntibodies see platelets as bacteria and work to eliminate themITP is preceded by a viral illnessITP is preceded by a viral illness

URIVaricella / measles vaccineMononucleosisFlu

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Symptoms

Random purpuraEpistaxis, hematuria, hematemesis, and menorrhagiaPetechiae and hemorrhagic bullae in mouthPetechiae and hemorrhagic bullae in mouth

Diagnostic Tests

Low platelet count Peripheral blood smear Antiplatelet antibodies

Normal platelet count: 150,000 to 400,000

Management

IV gamma globulin to block antibody production, reduce autoimmune problemCorticosteroids to reduce inflammatory processprocessIV anti-D to stimulate platelet production

Sickle Cell Anemia

Autosomal recessive disorderDefect in hemoglobin moleculeCells become sickle shaped and rigidL bilit t d t h t diLose ability to adapt shape to surroundings.Sickling may be triggered by fever and emotional or physical stress

Pathophysiology

When exposed to diminished levels of oxygen, the hemoglobin in the RBC develops a sickle or crescent shape; the cells are rigid and obstruct capillary blood flow, leading to p y , gcongestion and tissue hypoxia; clinically, this hypoxia causes additional sickling and extensive infarctions.

Whaley & Wong Text

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Crescent Shaped Cells Body Systems Affected by SS

Brain: CVA – paralysis - deathEyes: retinopathy – blindnessLungs: pneumoniaAbdomen: pain, hepatomegaly, splenomegaly p , p g y, p g y(medical emergency due to possible ruptureSkeletal: joint pain, bone pain – osteomyelitisSkin: chronic ulcers – poor wound healing

Vaso-occlusive Crisis

Stasis of blood with clumping of cell in the microcirculation, ischemia, and infarctionMost common type of crisis; painfulSigns include fever pain tissue engorgementSigns include fever, pain, tissue engorgement

Splenic Sequestration

Life-threatening / death within hoursPooling of blood in the spleenSigns include profound anemia, hypovolemia, and shockand shockAbdominal distention, pallor, dyspnea, tachycardia, and hypotension

Aplastic Crisis

Diminished production and increased destruction of red blood cellsTriggered by viral infection or depletion of folic acidfolic acidSigns include profound anemia, pallor

Management - Hospital

Increase tissue perfusionOxygenBlood transfusion if orderedBed rest

Pain management Hydration

IV fluids as orderedOral intake of fluids

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Management

• Adequate nutrition• Emotional Support• Discharge instructions

• Information about disease management• Daily folic acid• Control of triggers• Prophylactic antibiotics • Immunizations / Pneumococcal

Patient Education

Necessity of following plan of careSigns and symptoms of impending crisis.Signs and symptoms of infectionP ti h i f h i l dPreventing hypoxia from physical and emotional stressProving adequate rest

Beta-Thalassemia

Hereditary / autosomal defectGenetic defect on chromosome 11Mediterranean descentD f t i th b t l biDefect in the beta globin geneBeta globin chains are required for synthesis of hemoglobin A

RBC Characteristics

Microcytosis = small in size

Hypochromia = decreaseHypochromia = decrease hemoglobin

Poikilocytosis = abnormal shape

Treatment / Prognosis

Supportive Blood transfusions as neededBone marrow transplant

Poor prognosis / death within 1st year due to septicemia orheart failure.

Iron Deficiency Anemia

Most common nutritional deficiencyDepletion of iron stores

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Abnormal Laboratory Values

Hemoglobin levels less than 8 g/dL

Decreased levels of Serum Iron or Total Iron Binding or Serum FerritinBinding or Serum Ferritin

Microcytic and hypochromic red blood cells

IDA

Occurs in children experiencing:Rapid physical growthLow iron intakeInadequate iron absorptionInadequate iron absorptionLoss of blood

Symptoms

Associated with low oxygenation of tissue:PallorFatigueShortness or breathShortness or breathIrritabilityIntolerance of physical work / exercise

Management

Iron supplementationGiven in a.m. on an empty stomachTo avoid staining of teeth, give using a syringe, dropper or strawppInstruct caretaker that child may have dark-colored stools

Management

Nutritional counselingInfants younger than 12 months should be on formula until around 12 months of ageInfants 12 months or older

Decrease intake of milk Introduce solid foods

Children: iron fortified cereals, foods, meat, green leafy vegetablesTeenagers: reduce junk food

Aplastic Anemia

Acquired or inherited

Normal production of blood cells in the bone marrow is absent or decreasedmarrow is absent or decreased.

A marked decrease in RBC’s, WBC’s and platelets.

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Causes

Exposure to drugsExposure to chemicalsExposure to toxinsI f tiInfectionIdiopathic in nature

Blood Characteristics

Neutophil less than 500 Platelet less than 20,000Hemoglobin less thanHemoglobin less than 7Reticulocytes 1%

Nursing Diagnosis?

Bone marrow reveals hypo-cellular and fatty marrow.

Management

Immunosuppressive therapy

Antithymocyte globulinAdministered IV over 4 daysAdministered IV over 4 daysResponse seen within 3 months

Bone Marrow Transplant

Hyper-bilirubinemia

Hyperbilirubinemia

Many babies have some jaundice. When they are a few days old, their skin slowly begins to turn yellow. The yellow color comes from the color of bilirubin. When red blood cells die, they break down and bilirubin is left. The red blood cells break down and make bilirubin. In newborns the liver may not be developed enough to getnewborns, the liver may not be developed enough to get rid of so much bilirubin at once. So, if too many red blood cells die at the same time, the baby can become very yellow or may even look orange. The yellow color does not hurt the baby's skin, but the bilirubin goes to the brain as well as to the skin. That can lead to brain damage.

Signs and symptoms

Very yellow or orange skin tones (beginning at the head and spreading to the toes) Increased sleepiness, so much that it is hard to wake the babyyHigh-pitched cryPoor sucking or nursingWeakness, limpness, or floppiness

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Photo Therapy Fiberoptic Blanket