large granular lymphocyte leukemia

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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