Hematology

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Hematology

• By
• Joyce Smith RN BSN

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Coagulation Disorder

• Platelet Disorders
• Thrombocytopenia
• Autoimmune Thrombocytopenia Purpura
• Thrombotic Thrombocytopenia Purpura
• Clotting Disorders
• Hemophilia
• DIC

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Autoimmune Thrombocytopenia Purpura

• Immune Thrombocytopenia Purpura
• Idiopathic Thrombocytopenia Purpura
• Immunologic platelet destruction causes a marked
  decrease in number of circulating platelets
• Most common acquired thrombocytopenia

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Autoimmune Thrombocytopenia Purpura

• Acute usually affects children 2-9, postviral
• Chronic adults lt50, F 20 to 40, six months
• Recovery occurs within 1 to 2 months for
  patients with acute 90
• Chronic 10 to 20 recover without treatment
• Risk of acute hemorrhage greatest during 1st
  2nd weeks, intracranial bleed fatal

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Risk Factors

• Immune-related disorders
• Viral infections such as rubella, chicken pox,
  mumps, measles, or smallpox
• Sensitivity to drugs, allergies, blood
  transfusion
• Exposure to insecticides chemicals, vinyl
  chloride

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Clinical Response

• Insidious onset of bleeding from the mouth, nose,
  and skin upon slight injury
• Spontaneous bleeding form mucous membranes
• Generalized weakness, fatigue, and lethargy,
  petechiae ecchymosis

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Assessment

• Spontaneous bleeding episodes, CBC with severely
  low platelets lt20,000/mm3
• Increased bleeding time, decreased platelet
  survival time possible platelet antibodies
• Question about exposure to chemicals, recent
  immunizations, exposure to or contractions of
  viral illness

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Assessment

• Assess for minor bleeding, epistaxis or bruising
  tendencies
• Look for petechiae, hematomas, superficial
  ecchymotic areas on skin
• Note change in LOC, confusion, lethargy
• Palpate abdomen for liver spleen enlargement

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Treatment

• Reduce control severity of bleeding
• Maintain homodynamic stability
• Identify possible cause of bleeding
• Plasmapheresis
• Splenectomy in chronic cases
• Platelet transfusion
• High-does gamma globulin to elevate platelet
  count reduce turnover

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Treatment

• Corticosteroids Imuran to suppress immune
  response in chronic
• Antimitotic drugs cyclophosphamide

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Nursing Diagnosis

• Risk for injury d/t prolonged bleeding time
• Control localized bleeding
• Transfuse if nec
• Teach adequate oral hygiene, including use of
  soft toothbrush or sponge, frequent brushing, no
  floss
• Avoid drugs that decrease platelet aggregation

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Interventions

• Caution patient to avoid using razors with blades
• Use normal saline nasal drops or sprays to
  decrease drying of mucous membranes

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Thrombotic Thrombocytopenia Purpura

• Rare blood condition characterized by formation
  of small clots in the circulation
• Uses up platelets causing low platelet count
• 1-3 million per year
• Most common 20-40 years old
• F 2X gt M
• Develops spontaneously lt20 factors that
  predispose

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A P

• Clots form in circulation temporarily disrupt
  local blood supply
• Affects the blood vessels of the brain kidney
• Headache
• Confusion
• Difficulty speaking
• Transient paralysis, numbness
• Hypertension

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Possible Causes

• Drugs
• Pregnancy
• Infections
• Systemic lupus erythematosus
• Malignancy

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Clinical Manifestations

• Malaise
• Fever
• Headache
• Occasionally diarrhea
• Bruising, rarely bleeding
• Purpura
• Ecchymosis

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Diagnosis

• No specific test to diagnose
• Symptoms
• Blood count
• Renal function
• Other illnesses with low platelet counts have to
  be excluded

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Treatment

• Corticosteroids
• RBC transfusions
• Folic acid
• Platelet transfusions
• Hepatitis B Vaccinations
• Aspirin may be started when platelet count
  reaches about ½ normal

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Treatment

• Plasma exchanges daily X 5 days to be effective,
  3 hours
• Plasma removed replaced by donor plasma
• Removes circulating antibodies against cleaving
  protease, plasma with normal cleaving protease
  activity
• Allergic reactions
• Tingling of fingers or around mouth caused by low
  calcium levels

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Poorly Responding Disease

• Alternative plasma replacement
• Vincristine
• Splenectomy
• Immunosuppression
• Azathioprine
• Cyclophosphamide
• Ciclosporin

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DIC (Disseminated Intravascular Coagulation)

• Hypercoagulability state
• Occurs when the bodies coagulation is
  overstimulated
• Secondary to sepsis, burns, cancer, major
  trauma, obstetric complications, CABG
• Diagnosis is by PTT, PT, thrombin time,
  fibrinogen level, and D-dimer
• May use heparin, administration of blood products
• Takes lots of time and persistence to overcome
  this problem

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Hemophilia

• Bleeding time greatly increased d/t impaired
  coagulation
• 0.01 of US population
• Hemophilia A deficient or absent factor VIII 80
  of cases
• Hemophilia B Christmas disease, deficient factor
  IX 15 of cases

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Risk Factors

• 30 of persons with hemophilia have on notable
  family history of disease
• Male
• Mother who is carrier, inherited x-linked
  recessive disorder, female carrier has 50 chance
  of transmitting X chromosome to son or daughter

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A P

• Clotting factor deficiency impairs the hemostatic
  response, preventing clot formation
• Severity varies with degree of clotting factor
  deficient, specific cause, location of bleeding

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Clinical Manifestations

• Spontaneous bleeding
• Skin musculoskeletal sites stressed or receive
  direct trauma
• Excessive bleeding after circumcision
• Prolonged bleeding after dental, surgical
  procedures, or childbirth
• Subcutaneous or muscular hematomas lead to
  pressure on vital organ produce damage

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Complications

• Significant of individuals with hemophilia
  received blood transfusions before 1984 became
  infected with HIV
• Bleeding into joints knees, ankles, elbows
  most common
• Repeated episodes lead to destruction of joint
  loss of motion

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Clinical Management

• Immediate halt bleeding
• Avoid aspirin for pain
• Immobilize joint apply ice
• Surgical correction of musculoskeletal
  complications
• Genetic counseling

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Interventions

• May be necessary to give transfusion prior to
  dental or surgical procedure
• Synovectomy, joint debridement, or arthroplasty
  to treat hemarthrosis complications

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Interventions

• Transfuse with cryoprecipitate, VIII
  freeze-dried concentrates VIII or IX
• Genetically engineered synthetic factor VIII or
  recombinant factor VIII
• Desmopressin for mild hemophilia

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Multiple Myeloma

• Neoplastic disease bone bone marrow
  infiltrated by defective plasma cells that form
  multiple tumors
• gt50, M 2X gtF, AAgtC 21
• Possible viral , hypersensitivity reaction,
  chronic inflammation, maybe genetic
• Excessive neoplastic plasma cells

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Clinical Manifestations

• Develop insidious slow
• Severe skeletal pain usually pelvis, spine,
  ribs
• Excessive accumulations of abnormal plasma cells
  in bone marrow
• Osteoporotic lesions in skull, vertebrae ribs

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Clinical Manifestations

• Degeneration of bones leads to calcium loss into
  serum cause hypercalcemia
• Precipitates renal dysfunction
• Anorexia
• Confusion
• Hyperuricemia

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Clinical Manifestations

• Thrombocytopenia
• Anemia
• Granulocytosis
• Fatigue
• Weakness
• Weight loss
• Tingling or myalgia of extremities

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Diagnostics

• Pancytopenia
• Elevated serum protein, hypocalcaemia,
  hyperuricemias creatinine
• Bence Jones protein in urine
• X-ray bone scan, MRI osteoporosis,
  demineralization, tumors
• Bone marrow aspirate biopsy

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Clinical Management

• Long-term symptoms management of chronic disease
• Control pain
• Palliative radiation
• Drugs to prevent complications
• Hypocalcaemia, hyperuricemia, dehydration

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Clinical Management

• Orthopedic support
• Plasmapheresis
• Chemotherapy
• BMT
• Fluids, diuretics, anti gout agents
• NSAID
• Skin care for RT

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Clinical Management

• Long-term prognosis is poor
• Final stages do not respond to treatment
• Hospice care