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1、徐运孝,内科学多媒体教案,HEMOLYTIC ANEMIAS,一、General Information, Hb Structure Definition of HemolyticAnemias,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
2、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,四、Di
3、agnosis,（一）Dominant evidence of diagnosis 1、Clinical manifestations of hemolytic anemia 2、Laboratory examinations show the lifespan 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
4、 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
5、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 compensatoryacceleration of
6、erythropoiesis,（2）Examination about the increased plasma free hemoglobin,moderate hemolysis combine with Hp Hp small part combine with hemopexin hemopexin free Hb albuminsevere hemolysis some convert to methemoglobin methemalbuminrenal reabsorption epithliummajor port hemoglobinuriaby renal tubuleur
7、inehemosiderin hemosiderineuriadesquamation,a free Hb b Hp c methemalbumin d Hb urine e urine rous test (+),（3）Examination about abnormal metabolism of bilirubin,Indirect bilirubinUrine urobilinogen，urine bilirubin（-）Stool bilinogen,2、Evidences of compensatory acceleration of erythropoiesis （1）Retic
8、ulocytosis （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 abo
9、ut hemoglobin synthesis abnormality Examinations about immunehemolysis,1、Examinations about red cell membrane,Erythrocyte osmotic fragility defectAutohemolysis test and its correctedtestAcidified serum hemolytic testGlucose water hemolytic test,2、Exminations about red cell enzyme abnormality,Autohem
10、olysis test and its corrected testHeinz body generation testMethemoglobin reduction testEnzyme activity test,3、Examinations about hemoglobinsynthesis abnormality,Hb electrophoresisHbF detectionEnclosion body generation testIsopropanol stability testHeinz body generation test,4、Examination about immu
11、ehemolysis,Coomb s test,五、Differential diagnosis,1、MDS、erythroleukemia,bone marrowmetastasis 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 Sple
12、nectomy 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 a
13、bnormal 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、DefinitionThe autoimmune hemolytic anemias ar
14、e 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 autoantibodies2、The destruction of the sensitized red cells by macrophages,三、Clinical manifestation,Extravascular hemolysis;chronic procedure;acute broken Evans syndrome,四、Diagnosis,Clinical manifestationCoombs test (+)Exclusion of other hemolyticanemias,
