英文溶血性贫血徐运孝__培训课件.pptVIP

（一）Get rid of the etiology,  treat the underlying  disease， （二）End hemolysis1、Glucocorticoid2、Immunosuppressive agents3、Plasma exchange4、Dextran （三）Correct anemia1、Compenont transfusion.Correct anemia and  ameliorate symptoms ,reduce the production of abnormal red  cells,relieve outside marrow  hematopoiesis,prevent bone changes and physical retardation2、Other drugs stimulating hematopoiesis （四）Splenectomy （五）Correct defect 1、Allo-BMT 2、Gene therapy  （六）Prevent complications Autoimmune Hemolytic Anemia 一、Definition The autoimmune hemolytic anemias are a group of disorders in which autoantibodies against antigens on the erythrocyte membrane cause a shortened red blood cell life span。 二、Pathogenesis 1、Production of autoantibodies 2、The destruction of the sensitized red cells by macrophages 三、Clinical manifestation Extravascular hemolysis;chronic procedure;acute broken Evan’s syndrome 四、Diagnosis Clinical manifestation Coombs test (+) Exclusion of other hemolytic anemias 五、Therapy Treatment of the cause Glucocorticoid Splenectormy Immunsuppressive agents Plasma exchange Paroxysmal Nocturnal Hemoglobinuria（PNH） 一、Definition PNH is an acquired hemopoietic stem cell disorder with red cell membrane defect,the classic abnormality is increased sensitivity to complement-mediated intravascular hemolysis. The clnical presentations include paroxysmal hemoglobinuria associated with sleep,may be pancytopenia and/or thrombosis 二、Pathogenesis Deletion of some proteins on the cell surface Production of GPI decreased PIG-A gene mutation Clonal disease 三、Clinical Manifextations （一）Intravascular hemolysis 1、Developed at first time、hemoglobinuria 2、Pancytopenia 3、Bone marrow examination 4、Relationship with AA （二）Complication Bilestone Thrombosis Others 四、Diagnosis 1、Clinical manifestation asso