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1、LargeGranularLymphocyteLeukemia论文.freelirSokola,ThomasP.Loughran,Jr.b【关键词】T-cell,LGL,leukemia,#8226;,Aggressive,NK-cell,leukemia,#8226;,Cytopenia,#8226;,AutoimmunityLEARNINGOBJECTIVESAfterpletingthiscourse,.freelolecularandcellularbiologyofLGLleukemia.Describedist
2、inctclinicalentitiesamongdisordersofLGLs.DiscussthediagnosticcriteriaforT-cellLGLleukemia.DiscussthetherapeuticalgorithmofLGLleukemia.ABSTRACTClonaldisordersoflargegranularlymphocytes(LGLs)representaspectrumofbiologicallydistinctlymphoproliferativediseasesoriginat
3、ingeitherfrommatureTcells(CD3+)ornaturalkiller(NK)cells(CD3).Bothsubtypes,T-cellandNK-cellLGLleukemia,canmanifestasindolentoraggressivedisorders.Themajorityofpatientsiahaveaclinicallyindolentcourseediansurvivaltime10years.Immunosuppressivetherapyethotrexate,cyclop
4、hosphamide,orcyclosporineAcancontrolsymptomsandcytopeniasinmorethan50%ofpatients,butthisapproachisnotcurative.Severalcasesofanaggressivevariant(CD3+CD56+)ofT-cellLGLleukemiaiaisusuallyarapidlyprogressivedisorderassociatederica.Thisdiseaseisusuallyrefractorytoconve
5、ntionalchemotherapy,ediansurvivaltimeof2months.ChronicNK-cellleukemia/lymphocytosisisarareEBV-negativedisorderalignantoriginofthissubtypeisuncertainbecauseclonalityisdifficulttodetermineinLGLsofNK-cellorigin.DEFINITIONANDCLASSIFICATIONLargegranularlymphocytes(LGLs
6、)represent10%15%ofthetotalperipheralbloodmononuclearcellsinnormaladults1.Themajorityofthesecells(85%)arederivedfromtheCD3naturalkiller(NK)celllineage,andaminorityarederivedfromtheCD3+T-celllineage(15%).Cytologically,LGLsaremediumtolargecellsic,antigen-primedcytoto
7、xicCD8+Tlymphocytes.NK-cellLGLsbelongtotheinnateimmunesystemajorhistopatibilityplex(MHC)restrictedcytotoxicity.LGLleukemiaarros3.ClonaldisordersofLGLsrepresentabiologicallyheterogeneousspectrumoflymphoidmalignancies(Fig.2)4,5.BoththeT-cellandNK-cellsubtypescanclin
8、icallypresentasanindolentoraggressivediseases(Table1).IOLOGYLGLleukemiaprises2%5%ofallT-cell/NK-cellmalignancies,iaisthemostmonsubtype,representingappro
