英文溶血性贫血徐运孝

徐运孝,内科学多媒体教案,HEMOLYTIC ANEMIAS,一、General Information, Hb Structure Definition of Hemolytic Anemias,1、Hb StructureH bA（ 2 2） H bA2（ 2 2）H bF （ 2 2 ） Protoporphyrin HematinH 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 disordersHemolytic jaundice,二、Classification and Pathogenesis1、Hemolytic sites: Extravascular hemolysis Intravascular hemolysis Hemolysis in situ2、Hereditary Tendency: Hereditary Acquired,3、PathogenesisIntrinsic abnormities of red cells: red cell membrane disorders ；enzyme defects ；hereditary Hb disease. Extrinsic abnormities of red cells: Immune；Physical and mechanical； chemical；infections。,三、Clinical Manifestations,四、Diagnosis,（一）Dominant evidence of diagnosis1、Clinical manifestations of hemolytic anemia2、Laboratory examinations show the life span of red cells is shortened and the destruction of red cells is excessive.3、Significant increase of normoblast hyperplasia in bone marrow,（二）Distinguish whether there is hemolytic anemia or not1、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,stomatocyte,helmet cell,sickle cell. LDH ,2、Evidence of compensatory acceleration of erythropoiesis,（2）Examination about the increased plasma free hemoglobin,moderate hemolysis combine with Hp Hp small part combine with hemopexin hemopexinfreeHb 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,Indirect bilirubin Urine urobilinogen，urine bilirubin（-） 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,Erythrocyte osmotic fragility defect Autohemolysis test and its corrected test Acidified serum hemolytic test Glucose water hemolytic test,2、Exminations about red cell enzyme abnormality,Autohemolysis test and its corrected test Heinz body generation test Methemoglobin reduction test Enzyme activity test,3、Examinations about hemoglobinsynthesis abnormality,Hb electrophoresis HbF detection Enclosion body generation test Isopropanol stability test Heinz body generation test,4、Examination about immuehemolysis,Coomb s test,五、Differential diagnosis,1、MDS、erythroleukemia,bone marrow metastasis2、Other proliferative anemia3、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 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-BMT2、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 brokenEvans 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 associated with PNH2、Laboratory examinations Blood routine examination Bone marrow cha