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 <50, 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 <20,000/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 > M • Develops spontaneously <20% 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 • >50, M 2X >F, AA>C 2:1 • 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