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IMMUNE THROMBOCYTOPENIA

IMMUNE THROMBOCYTOPENIA. Cathy Payne MSN, ACNP-BC Hematology/Oncology Nurse Practitioner Ironwood Cancer and Research Centers. OBJECTIVES. • Define ITP • Terminology • Epidemiology • Mortality/Morbidity • Pathophysiology • Diagnosis • ASH guidelines/Management.

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IMMUNE THROMBOCYTOPENIA

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  1. IMMUNE THROMBOCYTOPENIA Cathy Payne MSN, ACNP-BC Hematology/Oncology Nurse Practitioner Ironwood Cancer and Research Centers

  2. OBJECTIVES • Define ITP • Terminology • Epidemiology • Mortality/Morbidity • Pathophysiology • Diagnosis • ASH guidelines/Management

  3. Immune Thrombocytopenia • ITP is an autoimmune-mediated hematologic disorder, in which the patients immune system produces antibodies directed against platelet antigens resulting in immune mediated platelet destruction and/or suppression of platelet production • Cines, D.B., Blanchet, VS, (2002) Immune thrombocytopenia purpura.

  4. PATHOPHYSIOLOGY • reduced platelet lifespan due to antibody mediated destruction, as well as impaired platelet production • specific IgG autoantibodies produced by the patient’s B cells most often are directed against • platelet membrane glycoproteins like GPIIb/IIIa inciting events- viral infections and systemic conditions, malignancies

  5. TERMINOLOGY • Diagnostic platelet count <100,000 • Primary vs secondary • Duration • Severity • Refractory ITP • Response to treatment

  6. EPIDEMIOLOGY • 1 to 3 per 100,000 adults • Young women • Incidence increases with age with a slight male predominance

  7. MORBIDITY/MORTALITY • Most patients will have good outcomes with infrequent hospital admissions and no excess mortality compared with the general population • patients with persistent severe thrombocytopenia not responding to therapy within first 2 years have considerable M/M • Bleeding/infections cause of death

  8. DIAGNOSING ITP • HISTORY • ethanol history • liver disease • drug history • bleeding/bruising • family history • risk factors for HIV or hepatitis C and or B symptoms • Symptoms of MDS • autoimmune disorders • recent viral infection • history of transfusions

  9. PHYSICAL EXAM • petechiae • purpura • conjunctival bleeding • retinal hemorrhages • lesions on mucosal membranes • enlarged liver or spleen • lymph nodes • sighs of sepsis, DIC or other severe systemic abnormalities • pregnancy

  10. COMPLETE BLOOD COUNT • Isolated thrombocytopenia, plt count < 100,000 • anemia only if significant bleeding otherwise normal red cell indices, WBC count and differential, normal coagulation parameters • peripheral blood smear- schistocytes

  11. LAB TESTS • HIV and Hep C • coagulation studies • Helicobacter pylori • thyroid funtion • rheumatological studies • Vitamin B12 and folate levels • anti-platelet antibody testing- NO

  12. BONE MARROW EXAM • not always needed, but do if the etiology of thrombocytopenia is unclear • any abnormality with History/Physical, blood smear or blood indices • yes for those older than 60 years

  13. DIFFERENTIAL DIAGNOSIS • drug induced • infections • liver disease and hypersplenism • microangiopathic processes • MDS and other bone marrow disorders • vasculiticpurpura

  14. ASH GUIDELINES/MANAGEMENT OF ITP INITIAL MANAGEMENT • consider treatment for patients with a platelet count <30,000 • longer courses of corticosteroids are preferred over shorter courses of corticosteriods or IVIG • IVIG may be used in conjunction with corticosteriods if a more rapid increase in platelet count is required • either IVIG (1g.kg for one dose, repeated as necessary) or anti-D (in appropriate patients) may be used as a first line treatment if corticosteriods are contraindicated

  15. ASH GUIDELINES FOR SENCOND LINE THERAPY • splenectomy • rituximab • thrombopoientin receptor agonist • high dose dexamethasone • immunosuppression

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