CHAPTERAutoimmuneandIntravascularHemolyticAnemias
DEFINITION
Hemolytic[hi:m?litik]anemia(HA)溶血性贫血isdefinedasanemiacausedbyashortenedlifespanofmatureredbloodcells(RBCs)intheperipheralcirculation.Hemolysis[him?lisis]溶血andaccelerateddestructionofRBCscantakeplacewithinthevasculature(i.e.,intravascularhemolysis)ormainlyintheliverandthespleen(i.e.,extravascularhemolysis).HAcanbetheconsequenceofanintrinsicandoftengeneticallydetermineddefectoftheRBCmembraneoranRBCconstituent[k?nstitju?nt]成分(hemoglobin[Hb]structureorenzymemachinery),orHAcanresultfromanextrinsicandusuallyacquireddisorderoftheRBCmembrane(immune,infectious,toxic).
Autoimmunehemolyticanemia(AIHA)isanacquiredautoimmunediseaseinwhichautoantibodiesdirectedagainstautologous/??tɑl?ɡ?s/自体的RBCmembraneantigensleadtotheiraccelerateddestruction.ThediagnosisofAIHAisthusbasedonthepresenceofapositiveresultonthedirectantiglobulintest(DAT),alsoknownasthedirectCoombstest,andontheabsenceofanyotherhereditaryofacquiredcauseofhemolysis.InAIHA,hemolysisismainlyextravascular,butsomefeaturesofcon白癜风危害北京哪家医院皮肤科治白癜风好
