Anaplastic Lymphoma Kinase-Positive Large B-Cell Lymphoma An Underrecognized Aggressive Lymphoma英文文献资料.docVIP

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Anaplastic Lymphoma Kinase-Positive Large B-Cell Lymphoma An Underrecognized Aggressive Lymphoma英文文献资料.doc

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Anaplastic Lymphoma Kinase-Positive Large B-Cell Lymphoma An Underrecognized Aggressive Lymphoma英文文献资料

HindawiPublishingCorporation AdvancesinHematology Volume2012,ArticleID529572,6pages doi:10.1155/2012/529572 ReviewArticle AnaplasticLymphomaKinase-PositiveLargeB-CellLymphoma: AnUnderrecognizedAggressiveLymphoma ElizabethA.MorganandAlessandraF.Nascimento DepartmentofPathology,BrighamandWomen’sHospital,Boston,MA02115,USA CorrespondenceshouldbeaddressedtoAlessandraF.Nascimento,alessandrafn@ Received15October2011;Accepted10November2011 AcademicEditor:KikkeriN.Naresh Copyright?2012E.A.MorganandA.F.Nascimento. This is an open access article distributed under the Creative Commons AttributionLicense,whichpermitsunrestricteduse,distribution,andreproductioninanymedium,providedtheoriginalworkis properlycited. Anaplastic lymphoma kinase-(ALK-) positive large B-cell lymphoma (ALK+ LBCL) is a rare, aggressive tumor characterized by an immunoblastic or plasmablastic morphologic appearance, expression of ALK, CD138, CD45, EMA, and often IgA by immunohistochemistry, and characteristic chromosomal translocations or rearrangements involving the ALK locus. The morphologicandimmunophenotypicoverlapofthistumorwithotherhematologicandnonhematologicmalignanciesmayresult inmisdiagnosis.Thetumorhasbeenidenti?edinbothpediatricandadultpopulationsanddemonstratesamalepredominance. Presentationismostoftennodal,particularlycervical.Noassociationwithimmunocompromiseorgeographiclocationhasbeen recognized. The most common gene rearrangement is between clathrin and ALK (t(2;17)(p23;q23)), resulting in the CLTC- ALKchimericprotein,althoughotherfusionshavebeendescribed.Responsetoconventionalchemotherapyispoor.Therecent introductionofthesmallmoleculeALKinhibitor,crizotinib,mayprovideapotentialnewtherapeuticoptionforpatientswiththis disease. 1.Introduction amalepredominanceof3–5:1.Theproportionofpatients under age 18 has been found to comprise approximately 15–20% of the total. One con?rmed case occurring in a patientwiththehumanimmunode?ciencyvirus(HIV)has been reported [12]. In general, patients do