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ID:22495082
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页数:22页
时间:2018-10-29
《large granular lymphocyte leukemia》由会员上传分享,免费在线阅读,更多相关内容在学术论文-天天文库。
1、LargeGranularLymphocyteLeukemia LEARNINGOBJECTIVES Afterpletingthiscourse,thereaderolecularandcellularbiologyofLGLleukemia. DescribedistinctclinicalentitiesamongdisordersofLGLs. DiscussthediagnosticcriteriaforT-cellLGLleukemia. Discussthetherapeuticalgorithmo
2、fLGLleukemia. ABSTRACT Clonaldisordersoflargegranularlymphocytes(LGLs)representaspectrumofbiologicallydistinctlymphoproliferativediseasesoriginatingeitherfrommatureTcells(CD3+)ornaturalkiller(NK)cells(CD3).Bothsubtypes,T-cellandNK-cellLGLleukemia,canmanifestasin
3、dolentoraggressivedisorders.Themajorityofpatientsiahaveaclinicallyindolentcourseediansurvivaltime>10years.Immunosuppressivetherapyethotrexate,cyclophosphamide,orcyclosporineAcancontrolsymptomsandcytopeniasinmorethan50%ofpatients,butthisapproachisnotcurative.Sev
4、eralcasesofanaggressivevariant(CD3+CD56+)ofT-cellLGLleukemiaiaisusuallyarapidlyprogressivedisorderassociatederica.Thisdiseaseisusuallyrefractorytoconventionalchemotherapy,ediansurvivaltimeof2months.ChronicNK-cellleukemia/lymphocytosisisarareEBV-negativedisorderali
5、gnantoriginofthissubtypeisuncertainbecauseclonalityisdifficulttodetermineinLGLsofNK-cellorigin. DEFINITIONANDCLASSIFICATION Largegranularlymphocytes(LGLs)represent10%15%ofthetotalperipheralbloodmononuclearcellsinnormaladults[1].Themajorityofthesecells(85%)areder
6、ivedfromtheCD3naturalkiller(NK)celllineage,andaminorityarederivedfromtheCD3+T-celllineage(15%).Cytologically,LGLsaremediumtolargecellsic,antigen-primedcytotoxicCD8+Tlymphocytes.NK-cellLGLsbelongtotheinnateimmunesystemajorhistopatibilityplex(MHC)restrictedcytotoxic
7、ity. LGLleukemiaarros[3].ClonaldisordersofLGLsrepresentabiologicallyheterogeneousspectrumoflymphoidmalignancies(Fig.2)[4,5].BoththeT-cellandNK-cellsubtypescanclinicallypresentasanindolentoraggressivediseases(Table1).IOLOGY LGLleukemiaprises2%5%ofallT-cell/NK-cel
8、lmalignancies,iaisthemostmonsubtype,representingapproximately85%ofallcasesdiagnosedinMUNOPHENOTYPE LGLleukemiacellshaveamatureT-orNK-cellimmunophenotyp
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