原发性皮肤母细胞样套细胞淋巴瘤——病例报告

Primary cutaneous blastoid mantle cell lymphoma-case report.

作者信息

Estrozi Bruna, Sanches José A, Varela Paulo C S, Bacchi Carlos E

机构信息

Pathology Reference Laboratory, Botucatu, São Paulo, Brazil.

出版信息

Am J Dermatopathol. 2009 Jun;31(4):398-400. doi: 10.1097/DAD.0b013e31819d845a.

DOI:10.1097/DAD.0b013e31819d845a

PMID:19461249

Abstract

Mantle cell lymphoma (MCL) commonly involves extranodal sites, usually as a manifestation of disseminated disease. In rare cases, MCLs may arise as a primary tumor in the skin. Blastoid mantle cell lymphoma (BV-MCL) is a rare variant and has a more aggressive clinical course. The phenotype of BV-MCL is characterized as CD20+, CD5+, cyclin D1+, CD23-, and CD10-. Interphase fluorescence in situ hybridization shows a characteristic t(11;14) fusion pattern. We report a case of a BV-MCL arising in skin as primary cutaneous MCL with the characteristic immunophenotype and translocation.

摘要

套细胞淋巴瘤（MCL）常累及结外部位，通常是播散性疾病的一种表现。在罕见情况下，MCL可作为原发性肿瘤发生于皮肤。母细胞样套细胞淋巴瘤（BV-MCL）是一种罕见的变异型，具有更具侵袭性的临床病程。BV-MCL的表型特征为CD20+、CD5+、细胞周期蛋白D1+、CD23-和CD10-。间期荧光原位杂交显示特征性的t(11;14)融合模式。我们报告一例原发性皮肤MCL，表现为BV-MCL，具有特征性免疫表型和易位。