英文 溶血性贫血（徐运孝）ppt课件.pptVIP

徐运孝;一、General Information;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。; （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  T1/2?15 d (normal 25-32d)  ? changes of red cell morphology:spherocyte,ellipotocyte,stomatocyte,helmet cell,sickle cell.  ? LDH ??;;;;（2）Examination about the increased  plasma free hemoglobin; a free Hb? b Hp ? c methemalbumin d Hb urine e urine rous test (+);;（3）Examination about abnormal metabolism of bilirubin;2、Evidences of compensatory  acceleration of erythrop