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以孤立性骨病变形式出现的淋巴瘤。

Lymphoma presenting as a solitary bone lesion.

作者信息

Jones D, Kraus M D, Dorfman D M

机构信息

Department of Pathology, Brigham and Women's Hospital, Boston, Massachusetts 02115, USA.

出版信息

Am J Clin Pathol. 1999 Feb;111(2):171-8. doi: 10.1093/ajcp/111.2.171.

DOI:10.1093/ajcp/111.2.171
PMID:9930137
Abstract

Primary lymphoma of bone has characteristic clinical and radiologic manifestations; however, its histologic features and clinical outcome show considerable variability. The histologic and immunophenotypic features of 13 adult patients with lymphoma as a solitary bone lesion were compared with clinical outcome. All tumors studied were non-Hodgkin lymphoma of anaplastic or large cell type and included B-cell (9 cases), T-cell (3 cases), and null cell (1 case) phenotypes. All patients responded well initially to systemic chemotherapy (with or without radiotherapy); however, disease in 6 patients progressed or recurred, and 5 patients died of disease. Local disease progression was seen in 2 patients, with 4 patients experiencing relapse at distant sites. Expression of CD30, present in 7 cases, was associated with an anaplastic or large noncleaved histologic appearance. Absence of CD30 expression characterized 6 cases, including 4 with multilobate or cleaved morphologic features. Five of 6 cases that recurred were associated with CD30 expression, including 3 with anaplastic features. The 4 tumors with cleaved or multilobate nuclear morphologic features were associated with long disease-free survivals and may represent a distinct lymphoma subtype with a good prognosis.

摘要

原发性骨淋巴瘤具有特征性的临床和影像学表现;然而,其组织学特征和临床结局存在相当大的变异性。将13例以孤立性骨病变形式出现淋巴瘤的成年患者的组织学和免疫表型特征与临床结局进行了比较。所有研究的肿瘤均为间变性或大细胞型非霍奇金淋巴瘤,包括B细胞表型(9例)、T细胞表型(3例)和无标记细胞表型(1例)。所有患者最初对全身化疗(联合或不联合放疗)反应良好;然而,6例患者疾病进展或复发,5例患者死于该疾病。2例患者出现局部疾病进展,4例患者在远处部位复发。7例患者存在CD30表达,其与间变性或大的未分化组织学表现相关。6例患者缺乏CD30表达,其中4例具有多叶状或分化的形态学特征。复发的6例患者中有5例与CD30表达相关,其中3例具有间变性特征。4例具有分化或多叶状核形态学特征的肿瘤与较长的无病生存期相关,可能代表一种预后良好的独特淋巴瘤亚型。

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