Kumar S, Krenacs L, Otsuki T, Kumar D, Harris C A, Wellmann A, Jaffe E S, Raffeld M
Laboratory of Pathology, National Cancer Institute, Bethesda, MD 20892, USA.
Am J Clin Pathol. 1996 Jun;105(6):737-43. doi: 10.1093/ajcp/105.6.737.
Multiple lymphomatous polyposis (MLP), characterized by multiple polyps involving long segments of the gastrointestinal (GI) tract, is believed to represent GI involvement by mantle cell lymphoma (MCL), primarily based on its histologic and immunophenotypic similarities with MCL. However, rearrangement of the bcl-1 locus, the molecular lesion characteristic of MCL, has not been investigated in this group of patients. The authors evaluated the morphologic, immunophenotypic, and molecular features of 18 cases of MLP and 8 B-cell lymphomas involving the GI tract (including 6 MALT lymphomas). All MLP cases presented with GI disease, and were histologically similar to MCL. DNA extracted from formalin-fixed, paraffin-embedded tissue was analyzed for evidence of bcl-1 rearrangement by PCR, using chromosome 11 specific and consensus JH primers. Amplifiable DNA was obtained in 24 of 26 cases (16 of 18 MLP cases and 8 of 8 controls). bcl-1 rearrangement was detected in 6 of 16 cases (38%), subsequently confirmed by sequencing of the breakpoint region, and in 0 of 8 controls. Immunostaining for cyclin D1 was positive in 14 of 18 MLPs, including the 6 bcl-1 rearranged cases and negative in 6 of 6 evaluable controls. The detection of bcl-1 rearrangement and cyclin D1 expression in cases of MLP supports the view that MLP represents primary MCL, of the GI tract. These techniques may also be helpful in differentiating MLP from other GI lymphomas, particularly low grade lymphomas of MALT, when only small routinely fixed endoscopic biopsies are available.
多发性淋巴瘤性息肉病（MLP）的特征是胃肠道（GI）长段出现多个息肉，据信这代表套细胞淋巴瘤（MCL）累及胃肠道，主要基于其与MCL在组织学和免疫表型上的相似性。然而，尚未对这组患者进行bcl-1基因座重排的研究，而bcl-1基因座重排是MCL的分子病变特征。作者评估了18例MLP和8例累及胃肠道的B细胞淋巴瘤（包括6例黏膜相关淋巴组织淋巴瘤）的形态学、免疫表型和分子特征。所有MLP病例均表现为胃肠道疾病，组织学上与MCL相似。使用11号染色体特异性引物和共有JH引物，通过聚合酶链反应（PCR）分析从福尔马林固定、石蜡包埋组织中提取的DNA，以寻找bcl-1重排的证据。26例中有24例获得了可扩增DNA（18例MLP病例中的16例和8例对照中的8例）。16例中有6例（38%）检测到bcl-1重排，随后通过断点区域测序得到证实，8例对照中未检测到。18例MLP中有14例细胞周期蛋白D1免疫染色呈阳性，包括6例bcl-1重排病例，6例可评估对照中有6例呈阴性。在MLP病例中检测到bcl-1重排和细胞周期蛋白D1表达支持了MLP代表胃肠道原发性MCL的观点。当只有少量常规固定的内镜活检标本时，这些技术也可能有助于将MLP与其他胃肠道淋巴瘤区分开来，特别是黏膜相关淋巴组织的低级别淋巴瘤。
