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Refractory itp

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Refractory itp

  1. 1. Refractory ITP patients should fulfill 2 criteria. First, they should have failed splenectomy or have relapsed thereafter. Second, they should either exhibit severe ITP or have a risk of bleeding that in the opinion of the attending physician requires therapy
  2. 2. REFRACTORY ITP  Two criteria have to be fulfilled at the same time: the lack of response or relapse after splenectomy with severe ITP or a bleeding risk that needs treatment according to the GP  Temporary response to corticosteroids or to intravenous immunoglobulins does not exclude a refractory form
  3. 3. TERMINOLOGY DISEASE DURATION  Newly diagnosed (previously acute) < 3 months  Persistent 3 to 12 months  Chronic >12 months
  4. 4. Destruction of Platelets
  5. 5. CLINICAL FEATURES  Easy or excessive bruising (purpura) — your skin naturally bruises and bleeds more easily as you age, but this shouldn't be confused with ITP  Superficial bleeding into your skin that appears as a rash of pinpoint-sized reddish-purple spots (petechiae), usually on your lower legs  Prolonged bleeding from cuts  Spontaneous bleeding from nose  Bleeding gums, especially after dental work  Blood in urine or stools  Unusually heavy menstrual flow  Fatigue
  6. 6. Etiology of Thrombocytopenia Decreased Production  Dehydration, Vitamin B12 or folic acid deficiency  Leukemia or myelodysplastic syndrome  Decreased production of thrombopoietin by the liver in liver failure  Sepsis, systemic viral or bacterial infection  Dengue fever can cause thrombocytopenia by direct infection of bone marrow megakaryocytes, as well as immunological shortened platelet survival.
  7. 7. Increased destruction of Platelets  Thrombotic thrombocytopenic purpura  Hemolytic-uremic syndrome  Disseminated intravascular coagulation  Paroxysmal nocturnal hemoglobinuria  Antiphospholipid syndrome  Systemic lupus erythematosus  Post-transfusion purpura  Neonatal alloimmune thrombocytopenia  Splenic sequestration of platelets due to hypersplenism  Dengue fever has been shown to cause shortened platelet survival and immunological platelet destruction.  HIV-associated thrombocytopenia[5]  Gaucher's disease
  9. 9. Thrombopoietin Receptor Agonist Romiplostim:is a thrombopoiesis stimulating Fc-peptide fusion protein (peptibody) that is administered by subcutaneous injection. ELTROMBOPAG:is an orally-administered agent with an effect similar to that of romiplostim. It too has been demonstrated to increase platelet counts and decrease bleeding in a dose-dependent manner
  10. 10. Side effects of Splenectomy Splenectomy increases the risk of sepsis due to encapsulated organisms( S.Pneumoniae, Haemophilus Influenzae). Thus patient should be vaccinated with Pneumococcal,Hib and Meningococcal vaccine.
  11. 11. Agent Dose Acute (IV) Methylprednisolone 30 mg/kg; max 1 g IVIG 1 g/kg Anti-D 75 μg/kg Vincristine 0.03 mg/kg; max 1.5 mg Vinblastine 10 mg (∼ 0.2 mg/kg) Maintenance (po) Danazol 10 mg/kg; round to the nearest 200 mg; 400-800 mg/day in adults Azathioprine 2-2.5 mg/ kg; round to the nearest 50 mg; 100-200 mg/day in adults Refractory ITP COMBINED Drugs
  12. 12. Reference
  13. 13. THANK YOU