Adam Patrick, Katzenberger Tiemo, Seeberger Harald, Gattenlöhner Stefan, Wolf Juergen, Steinlein Claus, Schmid Michael, Müller-Hermelink Hans-Konrad, Ott German
Pathologisches Institut, Universität Würzburg, Universitätsklinikum Köln, Germany.
Am J Surg Pathol. 2003 Nov;27(11):1473-6. doi: 10.1097/00000478-200311000-00012.
Anaplastic large cell lymphomas are associated with the t(2;5)(p23;q35) chromosome translocation in 40% to 60% of cases, leading to a new chimeric gene NPM-ALK. NPM-ALK positive lymphomas are generally reported to be of either T cell or null phenotype. In this report, we describe a diffuse large B-cell lymphoma associated with the classic t(2;5) translocation and both nuclear and cytoplasmic expression of ALK. The tumor consisted of medium-sized to large immunoblasts and plasmablasts that on immunohistology were negative for CD30, CD20, and CD79a but showed monotypic cytoplasmic expression of lambda light chains. Clonality analysis confirmed B-cell lineage of the tumor cells. The t(2;5)(p23;q35) chromosome translocation was demonstrated as part of a complex karyotypic alteration by classic banding and spectral karyotyping (SKY) analyses. Reverse transcription polymerase chain reaction confirmed rearrangement of NPM and ALK genes. This case exemplifies that the t(2;5) can, albeit rarely, occur in large B-cell lymphomas and is not entirely limited to anaplastic large cell lymphomas of T or null cell phenotypes.
间变性大细胞淋巴瘤在40%至60%的病例中与t(2;5)(p23;q35)染色体易位相关,导致新的嵌合基因NPM-ALK。NPM-ALK阳性淋巴瘤通常报道为T细胞或无表型。在本报告中,我们描述了一例与经典t(2;5)易位以及ALK核和胞质表达相关的弥漫性大B细胞淋巴瘤。肿瘤由中等大小至大的免疫母细胞和成浆细胞组成,免疫组织化学显示其CD30、CD20和CD79a均为阴性,但显示λ轻链的单型胞质表达。克隆性分析证实肿瘤细胞为B细胞系。通过经典显带和光谱核型分析(SKY)证实t(2;5)(p23;q35)染色体易位是复杂核型改变的一部分。逆转录聚合酶链反应证实NPM和ALK基因重排。该病例表明,t(2;5)虽然罕见,但可发生于大B细胞淋巴瘤,并不完全局限于T细胞或无细胞表型的间变性大细胞淋巴瘤。