Hematology
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Hematology . Jan Bazner-Chandler CPNP, CNS, MSN, RN. Blood. Blood is the fluid of life Blood is composed of: Plasma RBC WBC Platelets. Plasma. Plasma consists of: 90% water. 10 % solutes: albumin, electrolytes and proteins.

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Hematology

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Hematology

Jan Bazner-Chandler

CPNP, CNS, MSN, RN


Blood

  • Blood is the fluid of life

  • Blood is composed of:

    • Plasma

    • RBC

    • WBC

    • Platelets


Plasma

  • Plasma consists of:

    • 90% water.

    • 10 % solutes: albumin, electrolytes and proteins.

    • 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 bright red in color.

  • Hemoglobin contains iron, making it an excellent vehicle for transporting oxygen and carbon dioxide.


Average life cycle is 120 days.

The bones are continually producing new cells.

RBC’s


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:

    • Produce protective antibodies.

    • Surround it and devour the bacteria.


WBC’s

  • WBC life span is from a few days to a few weeks.

  • WBC’s will increase when fighting infection.


Platelets are irregularly-shaped, colorless bodies that are present in blood.

Their sticky surface lets them form clots to stop bleeding.

Platelets


Blood Values

  • CBC with differential and platelet count.

    • Hgb:

      • Normal levels are 11 to 16 g / dl

      • Panic levels are:

        • Less than 5 g / dl

        • More 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 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 cells.

    • Red blood cells – carry oxygen to tissue

    • Platelets – help blood to clot

    • White blood cells – fight infection


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 – anemia

    • Platelets – platelet dysfunction

    • Fresh frozen plasma – coagulation factors


Blood Transfusions

  • 3 types of transfusion reactions

    • Hemolytic

    • Allergic

    • Febrile


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 can stimulate B lymphocytes to produce antibodies to the red blood cell antigens.


Hemolytic reaction

  • Flank pain

  • Fever

  • Chills

  • Bloody urine

  • Rash

  • Low blood pressure

  • Dizziness / fainting


Nursing Management

  • Stop the blood transfusion.

  • Start normal saline infusion.

  • Take vital signs with blood pressure

  • Call the MD

  • Obtain blood sample and urine specimen.

  • Return blood to blood bank.

  • Document


Febrile Reaction

  • Often occurs after multiple blood transfusions.

  • Symptoms:fever, chills, and diaphoresis.

  • Interventions:

    • Slow transfusion and administer antipyretic.

    • Administer antipyretic prior to administration.


Allergic Reaction

  • Symptoms: rash, urticaria, respiratory distress, or anaphylaxis.

  • Interventions:

    • administer antihistamine before transfusion

    • Physician may order washed rbc’s


Hematologic Conditions


Alteration in Hematologic Status

  • Disorders of hemostasis or clotting factors

  • Structural or quantitative abnormalities in the hemoglobin.

  • Anemias

  • Aplastic Anemia


Genetic Implications

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

    • Sickle cell anemia

    • Thalassemia

    • Hemophilia


Bleeding Disorders

  • Three types Hemophilia: males only

    • Type A most common – factor VIII deficiency

    • Type B - lack of factor IX (Christmas Disease)

    • Type 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 disorder

    • Affects 1 in 10,000 males in the US

    • Autoimmune disorder with lowered level of clotting factor

    • All races and socio economic groups affected equally


Hemophilia

  • Hemophilia is a sex-linked hereditary bleeding disorder

  • Transmitted on the X chromosome

  • Female is the carrier

  • Women 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 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.

    • Future: 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 trauma increases


Symptoms

  • May be mild, moderate or severe

  • Bleeding into joint spaces, hemarthrosis

  • Most dangerous bleed would be intracranial.


Diagnosis

  • Presenting symptoms

  • Prolonged activated aPTT and decreased levels of factor VIII or IX.

  • Genetic 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.


Nursing Diagnoses

  • Risk for injury

  • Pain with bleed especially into a joint

  • Impaired physical mobility

  • Knowledge deficit regarding disease and management of disease


Nursing interventions

  • No rectal temps.

  • Replace the factor as ordered by physician.

  • Manage pain utilizing analgesics as ordered.

  • Maintaining joint integrity during acute phase: immobilization, elevation, ice.

  • Physical therapy to prevent flexion contraction and to strengthen muscles and joints.

  • Provide opportunities for normal growth and development.


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 bracelet

  • Injury prevention appropriate for age

  • Signs and symptoms of internal bleeding or hemarthrosis

  • Dental checkups

  • Medication administration


Long Term Complications

  • 20% develop neutralizing antibodies that make replacement products less effective.

  • Gene therapy providing continuous production of the deficient clotting factor could be the next major advance in hemophilia treatment.


Disseminated Intravascular Coagulation or DIC

  • DIC is an acquired coagulopathy that is characterized by both thrombosis and hemorrhage.

  • 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, 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.


Nursing Diagnoses

  • Altered tissue perfusion

  • Risk for injury

  • Anxiety


Nursing Interventions

  • Rigorous ongoing assessment of all body systems

  • Monitor bleeding

  • No rectal temps

  • Avoid trauma to delicate tissue areas

  • All injections sites and IV sites need to be treated like an arterial stick.


Prognosis

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


ITP

  • Idiopathic thrombocytopenic purpura

    • Idiopathic = cause is unknown

    • Thrombocytopenic = blood does not have enough platelets

    • Purpura = excessive bleeding / bruising


Immune Thrombocytopenic Purpura

  • Antibodies destroy platelets

  • Antibodies see platelets as bacteria and work to eliminate them

  • ITP is preceded by a viral illness

    • URI

    • Varicella / measles vaccine

    • Mononucleosis

    • Flu


Symptoms

  • Random purpura

  • Epistaxis, hematuria, hematemesis, and menorrhagia

  • Petechiae 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 problem

  • Corticosteroids to reduce inflammatory process

  • IV anti-D to stimulate platelet production


Sickle Cell Anemia

  • Autosomal recessive disorder

  • Defect in hemoglobin molecule

  • Cells become sickle shaped and rigid

  • Lose 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 congestion and tissue hypoxia; clinically, this hypoxia causes additional sickling and extensive infarctions.


Whaley & Wong Text


Crescent Shaped Cells


Body Systems Affected by SS

  • Brain: CVA – paralysis - death

  • Eyes: retinopathy – blindness

  • Lungs: pneumonia

  • Abdomen: pain, hepatomegaly, splenomegaly (medical emergency due to possible rupture

  • Skeletal: joint pain, bone pain – osteomyelitis

  • Skin: chronic ulcers – poor wound healing


Vaso-occlusive Crisis

Stasis of blood with clumping of cell in the microcirculation, ischemia, and infarction

  • Most common type of crisis; painful

  • Signs include fever, pain, tissue engorgement


Splenic Sequestration

  • Life-threatening / death within hours

  • Pooling of blood in the spleen

  • Signs include profound anemia, hypovolemia, and shock

  • Abdominal distention, pallor, dyspnea, tachycardia, and hypotension


Aplastic Crisis

  • Diminished production and increased destruction of red blood cells

  • Triggered by viral infection or depletion of folic acid

  • Signs include profound anemia, pallor


Nursing Diagnoses

  • Altered tissue perfusion

  • Pain

  • Risk for infection

  • Knowledge deficit regarding disease process


Nursing Management - Hospital

  • Increase tissue perfusion

    • Oxygen

    • Blood transfusion if ordered

    • Bed rest

  • Pain management

  • Hydration

    • IV fluids as ordered

    • Oral intake of fluids


Nursing 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 care

  • Signs and symptoms of impending crisis.

  • Signs and symptoms of infection

  • Preventing hypoxia from physical and emotional stress

  • Proving adequate rest


Beta-Thalassemia

  • Hereditary / autosomal defect

  • Genetic defect on chromosome 11

  • Mediterranean descent

  • Defect in the beta globin gene

  • Beta globin chains are required for synthesis of hemoglobin A


Microcytosis = small in size

Hypochromia = decrease hemoglobin

Poikilocytosis = abnormal shape

RBC Characteristics


Treatment / Prognosis

  • Supportive

  • Blood transfusions as needed

  • Bone marrow transplant

Poor prognosis / death within 1st year due to septicemia or

heart failure.


Iron Deficiency Anemia

  • Most common nutritional deficiency

  • Depletion of iron stores


Abnormal Laboratory Values

  • Hemoglobin levels less than 8 g/dL

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

  • Microcytic and hypochromic red blood cells


IDA

  • Occurs in children experiencing:

    • Rapid physical growth

    • Low iron intake

    • Inadequate iron absorption

    • Loss of blood


Symptoms

  • Associated with low oxygenation of tissue:

    • Pallor

    • Fatigue

    • Shortness or breath

    • Irritability

    • Intolerance of physical work / exercise


Management

  • Iron supplementation

    • Given in a.m. on an empty stomach

    • To avoid staining of teeth, give using a syringe, dropper or straw

    • Instruct caretaker that child may have dark-colored stools


Management

  • Nutritional counseling

  • Infants younger than 12 months should be on formula until around 12 months of age

  • Infants 12 months or older

    • Decrease intake of milk

    • Introduce solid foods

  • Children: iron fortified cereals, foods, meat, green leafy vegetables

  • Teenagers: reduce junk food


Aplastic Anemia

  • Acquired or inherited

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

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


Causes

  • Exposure to drugs

  • Exposure to chemicals

  • Exposure to toxins

  • Infection

  • Idiopathic in nature


Neutophil less than 500

Platelet less than 20,000

Hemoglobin less than 7

Reticulocytes 1%

Nursing Diagnosis?

Blood Characteristics

Bone marrow reveals hypo-cellular and fatty marrow.


Management

  • Immunosuppressive therapy

    • Antithymocyte globulin

      • Administered IV over 4 days

      • Response 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 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 baby

  • High-pitched cry 

  • Poor sucking or nursing 

  • Weakness, limpness, or floppiness


Photo Therapy


Fiberoptic Blanket


Nursing Interventions

  • Monitor bilirubin levels

  • Assess activity level – muscle tone – infant reflexes

  • Encourage po intake: May need to supplement with formula if inadequate breastfeeding

  • Weight daily to assess hydration status

  • Monitor stools – amount and number

  • Cover eyes while under bili-lights

  • Facilitate parent - infant bonding

    Loss of moro or startle reflex can indicate possible brain damage due to Kernicterus


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