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伴有t(11;14)(q13;q32)的非霍奇金淋巴瘤:套区/中等大小淋巴细胞淋巴瘤的一个亚型?

Non-Hodgkin's lymphomas with t(11;14)(q13;q32): a subset of mantle zone/intermediate lymphocytic lymphoma?

作者信息

Leroux D, Le Marc'Hadour F, Gressin R, Jacob M C, Keddari E, Monteil M, Caillot P, Jalbert P, Sotto J J

机构信息

Department of Genetics, CHU Grenoble, La Tronche, France.

出版信息

Br J Haematol. 1991 Mar;77(3):346-53. doi: 10.1111/j.1365-2141.1991.tb08582.x.

DOI:10.1111/j.1365-2141.1991.tb08582.x
PMID:2012759
Abstract

We here describe 13 patients with non-Hodgkin's lymphoma (NHL) and a translocation t(11:14)(q13:q32). They were part of a series of 163 patients with NHL and an abnormal karyotype, serially referred to our institution between January 1984 and 1990. Patients with t(11:14) seem to present several common and interesting features. Males are more frequently affected than females, and old people more than young. They present at diagnosis with advanced disease and usually show involvement of epithelium and bone marrow. With respect to histologic diagnoses, these patients are usually considered to be of low-grade malignancies. However, most of them do very poorly, have short complete remission and frequent relapses whatever the treatment. As a whole, the median survival rate is rather low. The cytologic, histologic as well as the immunologic patterns tend to be uniform: tumours are composed of small cells and display features of mantle zone/intermediate lymphocytic lymphoma. They express high IgM and low IgD levels and more commonly bear Ig lambda light chains. They also express all pan-B antigens (except CD23) as well as the CD5 antigen, but usually lack the CD10. According to these characteristics, these tumours could be placed in between lymphocytic lymphomas (which usually express CD23) and follicular lymphomas (which commonly lack IgD and CD5 and bear CD10 as well as a t(14:18).

摘要

我们在此描述13例患有非霍奇金淋巴瘤(NHL)且有t(11:14)(q13:q32)易位的患者。他们是1984年1月至1990年间连续转诊至我们机构的163例核型异常的NHL患者系列中的一部分。患有t(11:14)的患者似乎呈现出一些共同且有趣的特征。男性比女性更易受累,老年人比年轻人更易受累。他们在诊断时表现为晚期疾病,通常显示上皮和骨髓受累。关于组织学诊断,这些患者通常被认为是低级别恶性肿瘤。然而,他们中的大多数预后很差,完全缓解期短且无论接受何种治疗都频繁复发。总体而言,中位生存率相当低。细胞学、组织学以及免疫模式往往较为一致:肿瘤由小细胞组成,表现为套区/中等淋巴细胞淋巴瘤的特征。它们表达高IgM和低IgD水平,更常见地带有Igλ轻链。它们还表达所有泛B抗原(除CD23外)以及CD5抗原,但通常缺乏CD10。根据这些特征,这些肿瘤可归为淋巴细胞淋巴瘤(通常表达CD23)和滤泡性淋巴瘤(通常缺乏IgD和CD5且带有CD10以及t(14:18))之间。

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