英文 溶血性贫血（徐运孝）ppt课件

徐运孝 内科学多媒体教案 HEMOLYTIC ANEMIAS 一、General Information Hb Structure Definition of Hemolytic Anemias 1、Hb Structure H bA（ 2 2） H bA2（ 2 2） H bF （ 2 2 ） Protoporphyrin Hematin H b Fe 2 Haptoglobin non-2 2、Definition of Hemolytic Anemias Hemolytic anemia is one kind of anemia in which the destruction of red cell is increased while the compensatory function of marrow hematopoisis is insufficient 。 Hemolytic disorders Hemolytic jaundice 二、Classification and Pathogenesis 1、Hemolytic sites: Extravascular hemolysis Intravascular hemolysis Hemolysis in situ 2、Hereditary Tendency: Hereditary Acquired 3、Pathogenesis Intrinsic abnormities of red cells: red cell membrane disorders ；enzyme defects ；hereditary Hb disease. Extrinsic abnormities of red cells: Immune；Physical and mechanical； chemical；infections。 （二）Distinguish whether there is hemolytic anemia or not 1、Evidence of excessive destruction of red cells （1）Examinations about the shortened life span and the excessive destruction of the red cells. （2） Evidence of the improved free hemoglobin level. （3）Examinations about the abnormal metabolism of serum bilirubin (1)examination about the shortened life span and excessive destruction of the red cells. The life span of the red cells is shortened. 32P-DFP or H-DFP T1/215 d (normal 25-32d) changes of red cell morphology:spherocyte,ellipotocyte,sto matocyte,helmet cell,sickle cell. LDH 2 2、Evidence of compensatoryEvidence of compensatory acceleration of erythropoiesis acceleration of erythropoiesis （2）Examination about the increased plasma free hemoglobin moderate hemolysis combine with Hp Hp small part combine with hemopexin hemopexin free Hb albumin severe hemolysis some convert to methemoglobin methemalbumin renal reabsorption epithlium major port hemoglobinuria by renal tubule urine hemosiderin hemosiderineuria desquamation a free Hb b Hp c methemalbumin d Hb urine e urine rous test (+) （3）Examination about abnormal metabolism of bilirubin l Indirect bilirubin l Urine urobilinogen，urine bilirubin（-） l Stool bilinogen 2、Evidences of compensatory acceleration of erythropoiesis （1）Reticulocytosis （2）Blood smear anisocytosis, polychromatophilia of red cells. Howoll-Jolly body. Cabot ring，nucleated red cells. （3）Hyperplasia bone marrow （三）Find the etiology of hemolysis anemia Examinations about red cell membrane defect Examinations about red cell enzyme abnormality Examinations about hemoglobin synthesis abnormality Examinations about immunehemolysis 1、Examinations about red cell membrane l Erythrocyte osmotic fragility defect Autohemolysis test and its corrected test Acidified serum hemolytic test l Glucose water hemolytic test 2、Exminations about red cell enzyme abnormality l Autohemolysis test and its corrected test l Heinz body generation test l Methemoglobin reduction test l Enzyme activity test 3、Examinations about hemoglobinsynthesis abnormality l Hb electrophoresis l HbF detection l Enclosion body generation test l Isopropanol stability test l Heinz body generation test 4、Examination about immuehemolysis l Coomb s test 五、Differential diagnosis 1、MDS、erythroleukemia,bone marrow metastasis 2、Other proliferative anemia 3、Congenital non-hemolytic jaundice, Gilbert syndrome，Crigler-Najjar syndrome 六、Treatment Get rid of the etiology,treat the underlying disease. End hemolysis Correct anemia Splenectomy Correct defect Prevent complications （一）Get rid of the etiology, treat the underlying disease， （二）End hemolysis 1、Glucocorticoid 2、Immunosuppressive agents 3、Plasma exchange 4、Dextran （三）Correct anemia 1、Compenont transfusion.Correct anemia and ameliorate symptoms ,reduce the production of abnormal red cells,relieve outside marrow hematopoiesis,prevent bone changes and physical retardation 2、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 lExtravascular hemolysis;chronic procedure;acute broken lEvans syndrome 四、Diagnosis l Clinical manifestation l Coombs test (+) l Exclusion of other hemolytic anemias 五、Therapy l Treatment of the cause l Glucocorticoid l Splenectormy l Immunsuppressive agents l 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 l Deletion of some proteins on the cell surface l Production of GPI decreased l PIG-A gene mutation l 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 l Bilestone l Thrombosis l Others 四、Diagnosis 1、Clinical manifestation associated with PNH 2、Laboratory examinations Blood routine examination