...
首页> 外文期刊>Journal of Leukocyte Biology: An Official Publication of the Reticuloendothelial Society >The immunophenotype of mast cells and its utility in the diagnostic work‐up of systemic mastocytosis
【24h】

The immunophenotype of mast cells and its utility in the diagnostic work‐up of systemic mastocytosis

机译:肥大细胞的免疫表型及其在系统性肥大细胞增多症诊断中的作用

获取原文
           

摘要

SMcomprisesaheterogeneousgroupofdisorders,characterizedbyanabnormalaccumulationofclonalMCsin1ormoretissues,frequentlyinvolvingtheskinandBM.Despitethefactthatmostadultpatients(90%)carrythesamegeneticlesion(D816VKITmutation),thediseasepresentswithmultiplevariantswithverydistinctclinicalandbiologicfeatures,adiverseprognosis,anddifferenttherapeuticrequirements.RecentadvancesinthestandardizationofthestudyofBMMCbyMFCallowedreproducibleidentificationandcharacterizationofnormal/reactiveMCsandtheirprecursors,aswellastheestablishmentofthenormalMCmaturationalprofiles.Analysisoflargegroupsofpatientsversusnormal/reactivesampleshashighlightedtheexistenceofaberrantMCphenotypesinSM,whichareessentialforthediagnosisofthedisease.Inturn,3clearlydistinctandalteredmaturation‐associatedimmunophenotypicprofileshavebeenreportedrecentlyinSM,whichprovidecriteriaforthedistinctionbetweenISMpatientswithMC‐restrictedandmultilineageKITmutation;thus,immunphenotypingalsocontributestoprognosticstratificationofISM,particularlywhenanalysisoftheKITmutationonhighlypurifiedBMcellsisnotroutinelyavailableinthediagnosticwork‐upofthedisease...
机译:SMcomprisesaheterogeneousgroupofdisorders,characterizedbyanabnormalaccumulationofclonalMCsin1ormoretissues,frequentlyinvolvingtheskinandBM.Despitethefactthatmostadultpatients(> 90%)carrythesamegeneticlesion(D816VKITmutation),thediseasepresentswithmultiplevariantswithverydistinctclinicalandbiologicfeatures,adiverseprognosis,anddifferenttherapeuticrequirements.RecentadvancesinthestandardizationofthestudyofBMMCbyMFCallowedreproducibleidentificationandcharacterizationofnormal / reactiveMCsandtheirprecursors,aswellastheestablishmentofthenormalMCmaturationalprofiles.Analysisoflargegroupsofpatientsversusnormal / reactivesampleshashighlightedtheexistenceofaberrantMCphenotypesinSM,whichareessentialforthediagnosisofthedisease.Inturn,3clearlydistinctandalteredmaturation-associatedimmunophenotypicprofileshavebeenreportedrecentlyinSM,whichprovidecriteriaforthedistinctionbetweenISMpatientswithMC-restrictedandmultilineageKITmutation;因此,immunphenotypingalsocontributestoprognosticstratificationofISM,杂色当无法对高纯度BM细胞进行KIT突变分析时,通常无法在该疾病的诊断工作中使用...

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱:kefu@zhangqiaokeyan.com

京公网安备:11010802029741号 ICP备案号:京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有
  • 客服微信

  • 服务号