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套细胞淋巴瘤:80例临床病理研究

Mantle cell lymphoma: a clinicopathologic study of 80 cases.

作者信息

Argatoff L H, Connors J M, Klasa R J, Horsman D E, Gascoyne R D

机构信息

Department of Pathology, University of British Columbia, Vancouver, Canada.

出版信息

Blood. 1997 Mar 15;89(6):2067-78.

PMID:9058729
Abstract

Mantle cell lymphoma (MCL) is a relatively uncommon yet distinct type of malignant lymphoma whose clinical and pathological characterization has been limited by the small numbers of cases published to date. We studied 80 cases of MCL seen at a single institution over 7 years to determine both clinical and pathological prognostic factors. The patients in this study were predominantly male (70%) and older (mean age, 63 years) and presented with advanced-stage disease (88%). Extranodal involvement was common. Median overall survival (OS) was 43 months. Except for performance status, prognosis was not significantly influenced by clinical prognostic factors. Histologically, MCL architecture was classified as diffuse (78%), nodular (16%), or mantle zone (6%); the OS among these groups was identical. Increased mitotic activity (>20 mitotic figures per 10 high power fields), blastic transformation, and peripheral blood involvement at diagnosis also predicted for a worse outcome, but bone marrow involvement did not. The presence or absence of a translocation t(11; 14) by cytogenetic analysis or a bcl-1 rearrangement by Southern analysis did not significantly predict outcome. In summary, this study of 80 cases of MCL highlights its distinctive clinicopathologic features and shows that increased mitotic activity, blastic morphology, and peripheral blood involvement at diagnosis are prognostically important factors.

摘要

套细胞淋巴瘤(MCL)是一种相对罕见但独特的恶性淋巴瘤类型,其临床和病理特征迄今因已发表病例数量较少而受到限制。我们研究了在一家机构7年间所见的80例MCL病例,以确定临床和病理预后因素。本研究中的患者以男性为主(70%)且年龄较大(平均年龄63岁),并呈现晚期疾病(88%)。结外受累很常见。中位总生存期(OS)为43个月。除了体能状态外,临床预后因素对预后没有显著影响。组织学上,MCL结构分为弥漫性(78%)、结节性(16%)或套区(6%);这些组之间的OS相同。有丝分裂活性增加(每10个高倍视野中有>20个有丝分裂象)、母细胞转化以及诊断时外周血受累也预示着预后较差,但骨髓受累并非如此。细胞遗传学分析显示的t(11;14)易位或Southern分析显示的bcl-1重排的有无对预后没有显著预测作用。总之,这项对80例MCL病例的研究突出了其独特的临床病理特征,并表明有丝分裂活性增加、母细胞形态以及诊断时外周血受累是预后的重要因素。

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