Mediastinal diffuse large-cell lymphoma with sclerosis. A clinicopathologic study of 60 cases.
Mediastinal diffuse large-cell lymphoma with sclerosis. A clinicopathologic study of 60 cases.
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纵隔弥漫性大细胞淋巴瘤伴硬化。
DOI：
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发表时间：
1986
影响因子：
5.6
通讯作者：
J. Rosai
中科院分区：
文献类型：
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作者：
T. Perrone;G. Frizzera;J. Rosai
对60例纵隔弥漫性大细胞淋巴瘤伴硬化（MDLLS）进行回顾性分析。研究组由43名女性和17名男性组成。85%的患者在初次诊断时年龄在35岁或以下。30例在诊断后至少1年（中位数：34.5个月）仍然存活，6例患病存活（中位数：13个月），20例死于播散性疾病（中位数：16.5个月），4例死于其他原因。对8名死于疾病的患者进行了完整的尸检。最常累及的胸外器官为淋巴结、肾脏、肝脏、胰腺、胃肠道和卵巢。五十六例按Lukes-Collins分类法分类：滤泡中心细胞型35例，免疫母细胞型T（IBT）13例，免疫母细胞型B（伊布）7例，伊布和结节硬化型霍奇金病混合型1例，无法分类者4例。免疫组化证实53例起源于淋巴网状细胞，其中4例无法分型，8例分型为B细胞肿瘤。不利的预后因素是诊断时年龄小于25岁，肿瘤在胸腔外，疾病复发，IBT或伊布肿瘤组织学。有利的迹象是对初始治疗的良好反应和明显的肿瘤硬化。MDLLS最常被误认为是恶性胸腺瘤、淋巴瘤和霍奇金病。有助于识别MDLLS的标准进行了讨论。
A retrospective analysis was conducted of 60 cases of mediastinal diffuse large-cell lymphoma with sclerosis (MDLLS). The study group consisted of 43 females and 17 males. Eighty-five percent were 35 years of age or younger at time of initial diagnosis. Thirty are alive and well at least 1 year after diagnosis (median: 34.5 months), six are alive with disease (median: 13 months), 20 died of disseminated disease (median: 16.5 months), and four died of other causes. Complete autopsy was performed on eight of the patients who died of disease. The most frequently involved extrathoracic organs were lymph nodes, kidney, liver, pancreas, gastrointestinal tract, and ovary. Fifty-six cases were classified according to the Lukes-Collins scheme: 35 were follicular center cell, 13 immunoblastic T (IBT), seven immunoblastic B (IBB), one a composite of IBB and nodular sclerosing Hodgkin's disease; four cases were unclassifiable. Lymphoreticular origin was proven immunohistochemically in 53 cases, including the four unclassifiable examples and eight cases typed as B-cell tumors. Unfavorable prognostic factors were age less than 25 years at diagnosis, tumor outside the thoracic cavity at presentation, disease recurrence, and IBT or IBB tumor histology. Favorable signs were good response to initial therapy and marked tumor sclerosis. MDLLS is most often mistaken for malignant thymoma, seminoma, and Hodgkin's disease. Criteria helpful for the recognition of MDLLS are discussed.