一名慢性淋巴细胞白血病患者的原发性皮肤边缘区B细胞淋巴瘤

Primary cutaneous marginal zone B-cell lymphoma in a patient with chronic lymphocytic leukaemia.

作者信息

Robak E, Jesionek-Kupnicka D, Robak T, Holub A, Wawrzyniak E, Bartkowiak J, Bednarek A, Constantinu M, Urbanska-Rys H

机构信息

Department of Dermatology, Medical University of Łodz, Łodz, Poland.

出版信息

Br J Dermatol. 2007 Sep;157(3):591-5. doi: 10.1111/j.1365-2133.2007.07923.x.

DOI:10.1111/j.1365-2133.2007.07923.x

PMID:17697078

Abstract

Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is a low-grade malignant lymphoma that presents in the skin with no evidence of extracutaneous localization at diagnosis. We present an 80-year-old woman with B-cell chronic lymphocytic leukaemia (CLL) who developed multifocal PCMZL lesions 14 months after CLL diagnosis. PCMZL was clonally similar to the original bone marrow (BM) CLL cells. The specific translocation t(14;18) (q32;q21) with breakpoints in IGH and BCL2 loci was found in a skin specimen, but was absent in BM and peripheral blood (PB) cells. In contrast, a 13q deletion was found in BM and PB CLL cells. The patient was treated with chlorambucil and complete response of PCMZL was achieved. To our knowledge this is the first patient with CLL in whom PCMZL has been diagnosed.

摘要

原发性皮肤边缘区B细胞淋巴瘤（PCMZL）是一种低度恶性淋巴瘤，其表现为皮肤病变，诊断时无皮肤外定位证据。我们报告一名80岁患有B细胞慢性淋巴细胞白血病（CLL）的女性，在CLL诊断14个月后出现多灶性PCMZL病变。PCMZL与原始骨髓（BM）CLL细胞克隆相似。在皮肤标本中发现了具有IGH和BCL2基因座断点的特异性易位t(14;18)(q32;q21)，但在BM和外周血（PB）细胞中未发现。相反，在BM和PB CLL细胞中发现了13q缺失。该患者接受了苯丁酸氮芥治疗，PCMZL获得完全缓解。据我们所知，这是首例诊断为PCMZL的CLL患者。