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1、Immunodeficiencydisease(IDD)Primary/Congenital&Secondary/AcquiredImmunodeficiency免疫系统中某一成分的缺失或功能不全而导致免疫功能障碍所引起的疾病.ImmunodeficiencycausedbydefectsinBandTcellactivationComponentsoftheimmunesystem&deficiencyEachofthefourcomponentsoftheimmunesystem(Tcells,Bcells,phagocytes,andcomplem
2、ent)hasitsdomainoffunctionimportanttoprotectionagainstcertainpathogens.Thesecomponentsareintimatelyintegratedintoaprogramofimmunedefensethatcouldbeseverelycompromisedifevenonewereabsentordeficient.DefectsinspecificimmunecomponentsTheoccurrenceofrepeatedorunusualinfectionsinapatie
3、ntisaprimaryindicationofimmunodeficiency.Althoughadeficiencymaycompromiseseveralcomponentsoftheimmunesystem,inmostinstancesthedeficiencyismorerestrictedandresultsinsusceptibilitytoinfectionbysomebutnotallmicrobes.Forexample,defectsinTcellstendtoresultininfectionswithintracellular
4、microbes,whereasthoseinvolvingothercomponentsresultinextracellularinfections.ClassificationofimmunodeficienciesImmunodeficienciesareeitherprimary(mostlycongenital/inherited),orsecondary(acquiredastheconsequencesofotherdiseasesandtheirtreatments).PrimaryImmunodeficiency（原发性免疫缺陷）Se
5、condaryImmunodeficiency（继发性免疫缺陷）C1,C4,C2Ab+AgC3转化酶C3,C5转化酶C5,C6,C7,C8,C9MAC膜攻击ActivationofComplementPrimary/congenital(Inherited)Immunodeficiency——ComplementPatientsdeficientincertaincomplementcomponents(especiallyC3)arepronetorecurrentinfectionswithencapsulatedorganisms(Pneumoco
6、ccus肺炎链球菌andStreptococcus链球菌)andNeisseria（奈瑟氏球菌）.OpsonizationofthesepathogensbyC3bisimportantfortheirremovalbyphagocytosis.Deficienciesinmembraneattackcomplex(MAC)componentsandincomplementregulatorymoleculesalsoresultinincreasedsusceptibilitytocertaininfectionsortoinflammation,re
7、spectively.ComplementDeficienciesComponentdeficientDisease/infectionsRegulatorycomponentsC1qinhibitorHereditaryangiedema（遗传性血管性水肿）Decayacceleratingfactor(DAF促衰变因子)Paroxysmalnocturnalhemoglobulinuria（阵发性血红蛋白尿）(lysisofredbloodcells)ComplementcomponentsC1,C2orC4Immunecomplexdisease;
8、C2deficiencyassociatedwithSLEC3Themostse
