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Hematology

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Title: Hematology


1
Hematology
  • By
  • Joyce Smith RN BSN

2
Coagulation Disorder
  • Platelet Disorders
  • Thrombocytopenia
  • Autoimmune Thrombocytopenia Purpura
  • Thrombotic Thrombocytopenia Purpura
  • Clotting Disorders
  • Hemophilia
  • DIC

3
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

4
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

5
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

6
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

7
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

8
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

9
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

10
Treatment
  • Corticosteroids Imuran to suppress immune
    response in chronic
  • Antimitotic drugs cyclophosphamide

11
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

12
Interventions
  • Caution patient to avoid using razors with blades
  • Use normal saline nasal drops or sprays to
    decrease drying of mucous membranes

13
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

14
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

15
Possible Causes
  • Drugs
  • Pregnancy
  • Infections
  • Systemic lupus erythematosus
  • Malignancy

16
Clinical Manifestations
  • Malaise
  • Fever
  • Headache
  • Occasionally diarrhea
  • Bruising, rarely bleeding
  • Purpura
  • Ecchymosis

17
Diagnosis
  • No specific test to diagnose
  • Symptoms
  • Blood count
  • Renal function
  • Other illnesses with low platelet counts have to
    be excluded

18
Treatment
  • Corticosteroids
  • RBC transfusions
  • Folic acid
  • Platelet transfusions
  • Hepatitis B Vaccinations
  • Aspirin may be started when platelet count
    reaches about ½ normal

19
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

20
Poorly Responding Disease
  • Alternative plasma replacement
  • Vincristine
  • Splenectomy
  • Immunosuppression
  • Azathioprine
  • Cyclophosphamide
  • Ciclosporin

21
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

22
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

23
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

24
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

25
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

26
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

27
Clinical Management
  • Immediate halt bleeding
  • Avoid aspirin for pain
  • Immobilize joint apply ice
  • Surgical correction of musculoskeletal
    complications
  • Genetic counseling

28
Interventions
  • May be necessary to give transfusion prior to
    dental or surgical procedure
  • Synovectomy, joint debridement, or arthroplasty
    to treat hemarthrosis complications

29
Interventions
  • Transfuse with cryoprecipitate, VIII
    freeze-dried concentrates VIII or IX
  • Genetically engineered synthetic factor VIII or
    recombinant factor VIII
  • Desmopressin for mild hemophilia

30
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

31
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

32
Clinical Manifestations
  • Degeneration of bones leads to calcium loss into
    serum cause hypercalcemia
  • Precipitates renal dysfunction
  • Anorexia
  • Confusion
  • Hyperuricemia

33
Clinical Manifestations
  • Thrombocytopenia
  • Anemia
  • Granulocytosis
  • Fatigue
  • Weakness
  • Weight loss
  • Tingling or myalgia of extremities

34
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

35
Clinical Management
  • Long-term symptoms management of chronic disease
  • Control pain
  • Palliative radiation
  • Drugs to prevent complications
  • Hypocalcaemia, hyperuricemia, dehydration

36
Clinical Management
  • Orthopedic support
  • Plasmapheresis
  • Chemotherapy
  • BMT
  • Fluids, diuretics, anti gout agents
  • NSAID
  • Skin care for RT

37
Clinical Management
  • Long-term prognosis is poor
  • Final stages do not respond to treatment
  • Hospice care
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