refractory itp

Download Refractory itp

Post on 07-Aug-2015

32 views

Category:

Documents

4 download

Embed Size (px)

TRANSCRIPT

  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
  8. 8. TREATMENT
  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 g/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 www.bloodjournal.org www.mayoclinic.org
  13. 13. THANK YOU