英文溶血性贫血徐运孝课件

徐运孝徐运孝 内科学多媒体教案 HEMOLYTIC ANEMIAS 一、一、General InformationGeneral Information Hb Structure Definition of Hemolytic Anemias 1 1、HbHb Structure Structure H bAH bA（ 2 2 2 2 ） H bAH bA 2 2 （ 2 2 2 2 ） H bF H bF （ 2 2 2 2 ） ） ProtoporphyrinProtoporphyrin HematinHematin H bH b FeFe 2 2 Haptoglobin Haptoglobin non-non- 2 2 2 2、Definition of Hemolytic AnemiasDefinition of Hemolytic Anemias Hemolytic anemia is one kind of Hemolytic anemia is one kind of anemia in which the destruction of red anemia in which the destruction of red cell is increased while the compensatory cell is increased while the compensatory function of marrow hematopoisis is function of marrow hematopoisis is insufficient insufficient 。 Hemolytic disordersHemolytic disorders Hemolytic jaundiceHemolytic jaundice 二、二、Classification and PathogenesisClassification and Pathogenesis 1 1、Hemolytic sitesHemolytic sites : : Extravascular hemolysis Extravascular hemolysis Intravascular hemolysis Intravascular hemolysis Hemolysis in situHemolysis in situ 2 2、Hereditary Tendency:Hereditary Tendency: Hereditary Hereditary AcquiredAcquired 四、四、DiagnosisDiagnosis （一）Dominant evidence of diagnosis 1、Clinical manifestations of hemolytic anemia 2、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）（二）Distinguish whether thereDistinguish whether there is hemolytic anemia or not is hemolytic anemia or not 1、Evidence of excessive destruction of red cells （1 1）Examinations about the shortened Examinations about the shortened life span and the excessive life span and the excessive destruction of the red cells. destruction of the red cells. （2 2） Evidence of the improved free of the improved free hemoglobin level. hemoglobin level. （3 3）Examinations about the abnormal Examinations about the abnormal metabolism of serum bilirubin metabolism of serum bilirubin (1)examination about the shortened (1)examination about the shortened life span and excessive destruction life span and excessive destruction of the red cells.of the red cells. The life span of the red cells isThe life span of the red cells is shortened. shortened. 32 32P-DFP or H-DFP P-DFP or H-DFP T T1/2 1/2 15 d (normal 25-32d) 15 d (normal 25-32d) changes of red cell changes of red cell morphology:spherocyte,ellipotocyte,stomorphology:spherocyte,ellipotocyte,sto matocyte,helmet cell,sickle cell. matocyte,helmet cell,sickle cell. LDH LDH 2 2、Evidence of compensatoryEvidence of compensatory acceleration of erythropoiesis acceleration of erythropoiesis （2 2）Examination about the increased Examination about the increased plasma free hemoglobin 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 a freefree Hb Hb b Hp b Hp c methemalbumin c methemalbumin d Hb urine d Hb urine e urine rous test ( e urine rous test (+ +) ) （3 3）Examination about Examination about abnormal metabolism of bilirubinabnormal metabolism of bilirubin l Indirect bilirubin l Urine urobilinogen，urine bilirubin（-） l Stool bilinogen 2 2、Evidences of compensatory Evidences of compensatory acceleration of erythropoiesis acceleration of erythropoiesis （1 1）ReticulocytosisReticulocytosis （2 2）Blood smearBlood smear anisocytosis,anisocytosis, polychromatophilia of red polychromatophilia of red cells. cells. Howoll-Jolly body. Cabot Howoll-Jolly body. Cabot ring ring，nucleated red cells.nucleated red cells. （3 3）Hyperplasia bone marrowHyperplasia bone marrow （三）（三）Find the etiology of Find the etiology of hemolysis anemiahemolysis anemia Examinations about red cell membrane defect Examinations about red cell enzyme abnormality Examinations about hemoglobin synthesis abnormality Examinations about immunehemolysis 1 1、Examinations aboutExaminations about red cell membrane red cell membrane l Erythrocyte osmotic fragility defect Autohemolysis test and its corrected test Acidified serum hemolytic test l Glucose water hemolytic test 2 2、Exminations about redExminations about red cell enzyme abnormality cell enzyme abnormality l Autohemolysis test and its corrected test l Heinz body generation test l Methemoglobin reduction test l Enzyme activity test 3 3、Examinations about Examinations about hemoglobinsynthesis abnormality hemoglobinsynthesis abnormality l Hb electrophoresis l HbF detection l Enclosion body generation test l Isopropanol stability test l Heinz body generation test 4 4、Examination about Examination about immuehemolysis immuehemolysis l Coomb s test 五、五、Differential diagnosisDifferential diagnosis 1、MDS、erythroleukemia,bone marrow metastasis 2、Other proliferative anemia 3、Congenital non-hemolytic jaundice, Gilbert syndrome，Crigler-Najjar syndrome 六、六、TreatmentTreatment Get rid of the etiology,treat the underlying disease. End hemolysis Correct anemia Splenectomy Correct defect Prevent complications （一）（一）Get rid of the etiology, Get rid of the etiology, treat the underlying treat the underlying disease disease， （二）（二）End hemolysisEnd hemolysis 1 1、GlucocorticoidGlucocorticoid 2 2、Immunosuppressive agentsImmunosuppressive agents 3 3、Plasma exchangePlasma exchange 4 4、DextranDextran （三）（三）Correct anemiaCorrect anemia 1 1、CompenontCompenont transfusion.Correct anemia and transfusion.Correct anemia and ameliorate symptoms ,reduce ameliorate symptoms ,reduce the production of abnormal red the production of abnormal red cells,relieve outside marrow cells,relieve outside marrow hematopoiesis,prevent bone hematopoiesis,prevent bone changes and physical retardation changes and physical retardation 2 2、Other drugs stimulatingOther drugs stimulating hematopoiesis hematopoiesis （四）（四）SplenectomySplenectomy （五）（五）Correct defectCorrect defect 1、Allo-BMT 2、Gene therapy （六）Prevent complications Autoimmune Hemolytic AnemiaAutoimmune 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、二、PathogenesisPathogenesis 1、Production of autoantibodies 2、The destruction of the sensitized red cells by macrophages 三、三、Clinical manifestationClinical manifestation lExtravascular hemolysis;chronic procedure;acute broken lEvans syndrome 四、四、DiagnosisDiagnosis l Clinical manifestation l Coombs test (+) l Exclusion of other hemolytic anemias 五、五、TherapyTherapy l Treatment of the cause l Glucocorticoid l Splenectormy l Immunsuppressive agents l Plasma exchange Paroxysmal Nocturnal Paroxysmal Nocturnal HemoglobinuriaHemoglobinuria （PNHPNH） 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、二、PathogenesisPathogenesis l