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بـــسـم الله الرحـمـن الرحـيـم

بـــسـم الله الرحـمـن الرحـيـم. Review of Practice ITP Management. Salwa Hindawi MSc, MRCPath, CTM RCPE Director of Blood Transfusion Services King Abdulaziz University Hospital Jeddah, Saudi Arabia. PLATELETS. Produced from megakaryocytes in the bone marrow

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بـــسـم الله الرحـمـن الرحـيـم

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  1. بـــسـم الله الرحـمـن الرحـيـم Salwa Hindawi

  2. Review of Practice ITP Management Salwa Hindawi MSc, MRCPath, CTM RCPE Director of Blood Transfusion Services King Abdulaziz University Hospital Jeddah, Saudi Arabia Salwa Hindawi

  3. PLATELETS • Produced from megakaryocytes in the bone marrow • Mean survival: 8-10 days • Removed from circulation by cells of the monocyte-macrophage system • 1/3 of the total platelet mass is found in the spleen Salwa Hindawi

  4. Electron Micrscopy of Platelet Salwa Hindawi

  5. DEFINITION • <150,000 platelets/microliter (nml 150,000-400,000) • 2.5 % of the nml population have this • If platelet count >20,000, usually no serious spontaneous bleeding • If less than <10,000, risk of bleeding increases and may necessitate transfusion Salwa Hindawi

  6. MECHANISMS OF THROMBOCYTOPENIA • Decreased platelet production • Increased platelet destruction • Dilutional/distributional • Pseudothrombocytopenia Salwa Hindawi

  7. MEDICATIONS • Quinidine • Quinine • Rifampin • Bactrim • Methyldopa • Acetaminophen • Digoxin • Diclofenac • Amphotericin • Vancomycin • Amiodarone • Piperacillin • Sulfasalazine • Ethambutol • Lithium • heparin Salwa Hindawi

  8. MEDICATIONS • Methicillin • INH • Minoxidil • Nitroglycerine • Haldol • Tamoxifen • Diazepam • Gold Salwa Hindawi

  9. ITP • Autoimmune etiology (IgG antibody to platelets) • Etiology • 30% drug related • 30% underlying disease (connective tissue disorders, lymphoma, CLL) • 30% idiopathic • 10% viruses (HIV) • Platelets are hyperfunctional (spontaneous bleeding is rare) Salwa Hindawi

  10. Idiopathic Thrombocytopenic Purpura (ITP) • Autoimmune disease of children and adults • Sustained Low platelet count • No other causes )exclusion) Salwa Hindawi

  11. Pathophysiology Immune Mediated Mechanism  increase HLA-DR expression defects in cellular and humoral immunity  specific autoantibody production to GpIIb/IIIa and Gp16-IX Antigens Salwa Hindawi

  12. Pathophysiology Platelet  destruction  Low  bleeding + by platelet through mucus platelet acrophagescount membranes Auto Ab RES Salwa Hindawi

  13. Clinical Features of ITP in Children and Adults Feature Children Adults Occurrence Peak age (yr) 2-4 15-40 Sex (F:M) Equal 2:6:1 Presentation Onset Acute (most with symptoms < 1 week) Insidious (most with symptoms <2 months) Symptoms Purpura (<10% with severe bleeding) Purpura (typically bleeding not severe) Platelet count Most <20 x 109/L Most <20 x 109/L Course Spontaneous remission 83% 2% Chronic disease 24% 43% Response to splenectomy 71% 66% Eventual complete recovery 89% 64% Morbidity and mortality Cerebral hemorrhage <1% 3% Hemorrhagic death <1% 4% Mortality of chronic refractory 2% 5% disease Salwa Hindawi

  14. APPROACH TO DIAGNOSIS • HISTORY AND PHYSICAL EXAMINAION • Recent viral history • Diagnosed hematological disease • Family history • Recent live virus vaccination (measles) • Poor nutritional states • Medications • Pregnancy Salwa Hindawi

  15. Diagnosis Diagnosis by Exclusion 1 – Clinical finding Bleeding and /or purpura Isolated thrombocytopenia No evidence of other disease 2 – peripheral blood smear 3 – platelet antibody test Salwa Hindawi

  16. 4-Auto-immune Profile Antiphospholipid syndrome 5-B.M.A. in the presence of Atypical clinical features or no response to Rx better before initiation of steroid therapy Salwa Hindawi

  17. PERIPHERAL BLOOD SMEAR • “GOLD STANDARD” • Check for platelet clumping, platelet size, RBC morphology, presence of immature WBCs Salwa Hindawi

  18. Deferential Diagnosis • Spurious • Congenital Thrombocytopenia • Fanconi anaemia • Aplastic anaemia • Acute leukemia • Autoimmune diseases (SLE) • Hypersplenism • Microangiopathy Salwa Hindawi

  19. Management Salwa Hindawi

  20. Important Points to consider: • Observation & follow up • Let the treatment fit the patient (treat cases individually) • Evidence based medicine through randomized controlled trials and or clinical practice Salwa Hindawi

  21. Goal of the treatment • Not to achieve a normal platelet count but to prevent bleeding • 2 options: • Counseling and observation • or Rx if: low platelet and/or bleeding Salwa Hindawi

  22. Management of ITP • Prednisone1-2 mg/kg daily 60-80% achieve remission • Intravenous immunoglobulin 0.4 g/kg daily x 5 days, or 1 g/kg/d x 2 days • I.V. Anti-D 50-75 ug/kg High dose Dexamethasone 40mg/Kg x 4 days for 6 cycles Salwa Hindawi

  23. High dose methyle prednisolone (HDMP) Oral 7 days course (30 mg/kg/d/3 days then 20 mg/kg/d x 4 days) x 6 courses  platelet count by day 7 > 50 x 104/L • Splenectomy Introduced in 1916 Two thirds of patients achieve remission ineffective medical management or/and therapy associated toxicity Salwa Hindawi

  24. Medical therapy after failed splenectomy or refusal to allow splenectomy • Oral prednisone (dose as pre-splenectomy) • IVIg 0.8-1.0 g/kg; repeat once if platelet response at 48-72 hours inadequate • IV anti-D IV (Rh(D)-positive patients only), 50-75g/kg • Other (eg, azathioprine, cyclophosphamide, vincristine, danazol). Salwa Hindawi

  25. Emergency therapy • IV methylprednisolone 30 mg/kg/d (maximum, 1g) for 3 consecutive days • IVIg (1-2 g/kg over 2 days) • Platelet trasfusion Salwa Hindawi

  26. Rarely used therapies • Interferon- • Cyclosporine A • Combination chemotherapy • Plasma exchange • Staphylococcal protein A immunoadsorption • Dapsone • Ascorbic acid • Colchicine • Mabthera • Danazol Salwa Hindawi

  27. Immunosuppressive & Chemotherapy Azathioprine, cyclosporine Vincaalkaloid, cyclophosphamide Salwa Hindawi

  28. Platelet Transfusion • Evaluate the case • Life threatened emergency • IVIG Salwa Hindawi

  29. Follow-up • Clinical severity in addition to platelet count, CBC should be repeated within 7-10 days of diagnosis and only when there is a clinical indication or signs of resolution of clinical symptoms. Salwa Hindawi

  30. Conclusions • Learn from our mistakes • Observe the patients, treat only when it is really needed. • Treat individual cases. • Platelet transfusion is contraindicated • Evidence based medicine randomized control trials for management of ITP. Salwa Hindawi

  31. References • Immune Thrombocytopenic Purpura Let the treatment fit the patient, George, et al Editorial – New England Journal of Medicine January 13,2004. • Initial Treatment of Immune Thrombocytopenia Purpura with high-dose dexamethasone Cheng, et al, January 13, 2004. • Guidelines for the Investigation & Management of Idiopathic Thrombocytopenic Purpura in adult, children and in pregnancy, British Journal of Haematology 2003. Salwa Hindawi

  32. Pathogenesis and Management of Chronic Idiopathic Thrombocytopenic Purpura An Update, Renchiyang, Zhong Chao Han; International Journal of Haematology , Aug. 1999. • Blanchette V, Freedman J, Garvey B., Management of Chronic ImmuneThrombocytopenic Purpura in Children and Adults, Semin Haematol, 1998. • Idiopathic Thrombocytopenic Purpura: A Concise Summary of the Pathophysiology and Diagnosis in Children and Adults. James N. George & Gary E. Raskob; Seminars in Haematology 1998. • George JN, Woolf SH, Raskob GE, et al: Idiopathic Thrombocytopenic Purpura. A Practice Guideline Developed by Explicit Methods For American Society of Haematology Blood;88:3-40,1996. Salwa Hindawi

  33. Thanks Salwa Hindawi

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