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 1 st & 2 nd 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