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纵隔原发性大细胞淋巴瘤:20例分析

Primary large cell lymphomas of the mediastinum: an analysis of 20 cases.

作者信息

Waldron J A, Dohring E J, Farber L R

出版信息

Semin Diagn Pathol. 1985 Nov;2(4):281-95.

PMID:3879948
Abstract

Malignant lymphomas originating primarily in the mediastinum consist predominantly of Hodgkin's disease of the nodular sclerosis type, lymphoblastic lymphomas, and large cell non-Hodgkin's lymphomas of diffuse growth pattern (DHL). This analysis of 20 cases of primary mediastinal DHL presents the clinical and pathologic findings in nine patients with T-immunoblastic sarcoma (T-IBS), six with sclerosing variants of follicular center cell lymphoma (FCCL), and five with B-immunoblastic sarcoma (B-IBS). T-IBS patients were predominantly young adult women (mean age 31 years) presenting with relatively well confined mediastinal tumors; four of nine manifested the SVC syndrome. The immunomorphologic findings in T-IBS were similar to those of node-based peripheral T-cell lymphomas. Patients with FCCL and B-IBS were predominantly men, exhibited a broad age range, and presented with larger tumors with a high incidence of contiguous involvement of intrathoracic structures (83% in FCCL, 60% in B-IBS). Chemotherapeutic intervention attained CR in 19 of 20 patients, with 14 of 20 remaining alive in relapse-free CR a median of 26 months after completion of therapy. Durable CR was attained in eight of nine T-IBS patients, in four of six patients with FCCL, and in three of five patients with B-IBS. The morphologic features of these lymphoma subtypes are presented in detail and discussed in relation to the complex differential diagnosis of mediastinal neoplasms.

摘要

主要起源于纵隔的恶性淋巴瘤主要包括结节硬化型霍奇金病、淋巴母细胞淋巴瘤以及弥漫性生长模式的大细胞非霍奇金淋巴瘤(DHL)。本文对20例原发性纵隔DHL进行分析,呈现了9例T免疫母细胞肉瘤(T-IBS)、6例滤泡中心细胞淋巴瘤(FCCL)硬化变异型以及5例B免疫母细胞肉瘤(B-IBS)患者的临床和病理表现。T-IBS患者主要为年轻成年女性(平均年龄31岁),表现为纵隔肿瘤相对局限;9例中有4例出现上腔静脉综合征。T-IBS的免疫形态学表现与淋巴结外周T细胞淋巴瘤相似。FCCL和B-IBS患者主要为男性,年龄范围较广,肿瘤较大,胸腔内结构连续受累的发生率较高(FCCL为83%,B-IBS为60%)。20例患者中有19例通过化疗达到完全缓解(CR),20例中有14例在完成治疗后中位26个月仍无复发存活。9例T-IBS患者中有8例、6例FCCL患者中有4例以及5例B-IBS患者中有3例获得持久CR。详细介绍了这些淋巴瘤亚型的形态学特征,并结合纵隔肿瘤复杂的鉴别诊断进行了讨论。

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