Aplastic_Anemia_BJ贫血课件.ppt

APLASTIC ANEMIA;;Hematopoitic cell destruction by Immune Mechanisms in Acquired Aplastic Anemia. There is normal stromal cells but decreased number of colony forming cells and stem cells as it can be seen in Ps, radiographically, and also the stem cells number ; ; ; ;Etiology; ;Pathogenesis:;庚拄饯坐榆噎戮审户发搏锚过备略盔跃播撒疯匆逐敏康谜旁由憨酿闪租牧Aplastic_Anemia_BJ贫血课件Aplastic_Anemia_BJ贫血课件; ; ;Presentation of Anemia, Neutropenia and Thrombocytopenia;26-year-old woman with acute aplastic anemia and 1 day of facial pain/swelling. Mouth open involuntarily due to perioral edema. Needle aspirate of small purplish area near right alar revealed P aeruginosa.;Diagnosis and differential Diagnosis;Characteristic;Course and prognosis of the disease: with transfusion support alone: 80 % of cases succumb to death in 18 to 24 months, related to PMN count with MBT and Immunosuppresion: curative in 60 to 90 % of cases, risk of CGVH threat Supportive care: transfusion of the blood products, CMV seronegative should be given transfusion from the family members should be avoided to prevent sensitization. pooled donor platelets but leads to sensitization in refractory cases need HLA matched transfusion packed cells filtrated to remove leukocyte and platelets iron overload : give chelating therapy deferoxamine CMV prophylaxis Staph. Aeureus * hospitalization * menses ;Treatment modalities: ATG: purified monomeric IgG from hyperimuune horse with human thymocyte and thoracis duct lymphocytes 50 - 70 % good response 20-30 complete and durable recovery 70-80 have partial response Complication: MDS 30-6- % also secondary solid tumors response after 8 to 12 weeks Other drugs to suppress immunity: cyclosporine and cyclophosphamide and also high dose steroid Growth factors: Erythropoeitin, CG-CSF, GM-CSF, ILs 1,3, 6 ;Response is slow Response is often incomplete Response may require successive course of immunosuppression Relapse is common and require maintenance therapy Evolution to clonal disease is common: O