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B细胞型原发性纵隔透明细胞淋巴瘤

Primary mediastinal clear cell lymphoma of B-cell type.

作者信息

Möller P, Lämmler B, Eberlein-Gonska M, Feichter G E, Hofmann W J, Schmitteckert H, Otto H F

出版信息

Virchows Arch A Pathol Anat Histopathol. 1986;409(1):79-92. doi: 10.1007/BF00705408.

Abstract

This is a report on 8 mediastinal tumours that occurred in young adults (19-43 years, mean: 29.4); predominantly in females (6/8). Initial symptoms consisted of thoracic pain and venectasia and in only one case in B symptoms. After surgical tumour reduction, radiation and/or chemotherapy, local recurrence was observed in each case under clinical care; abdominal spread is presently suspected in 3 patients; 3 died 11, 13 and 22 months after diagnosis. None developed leukaemia. The tumours are B-cell neoplasms with a characteristic immunophenotype: leucocyte common antigen+, common acute lymphoblastic leukaemia antigen-, B 1-antigen+, surface and cytoplasmic immunoglobulin-. Flow cytometry revealed DNA-diploidy in 7 cases and a moderately (3.2-3.8%) to extremely high (8.0-20.6%) S-phase component. The proliferation associated antigen Ki67 was detectable in 10-60% of the tumour cell nuclei, thus stressing the considerable or rapid growth. Histopathology is characterized by a diffuse growth pattern and a clearness and abundance of cytoplasm of the pleomorphic tumour cells, which vary in size and nuclear morphology from patient to patient. Apoptoses are more numerous than mitoses. Fibrosis and focal necrosis are common, sclerosis is present in 3 cases. We suggest that primary mediastinal lymphoma of B cell type is a novel B-lymphoma variant.

摘要

这是一份关于8例发生在年轻人(19 - 43岁,平均29.4岁)的纵隔肿瘤的报告;主要发生在女性(6/8)。初始症状包括胸痛和静脉扩张,仅1例有B症状。在手术肿瘤减瘤、放疗和/或化疗后,临床护理下每例均观察到局部复发;目前怀疑3例有腹部转移;3例在诊断后11、13和22个月死亡。均未发生白血病。这些肿瘤是具有特征性免疫表型的B细胞肿瘤:白细胞共同抗原阳性、普通急性淋巴细胞白血病抗原阴性、B1抗原阳性、表面和细胞质免疫球蛋白阴性。流式细胞术显示7例为DNA二倍体,S期成分中等(3.2 - 3.8%)至极高(8.0 - 20.6%)。增殖相关抗原Ki67在10 - 60%的肿瘤细胞核中可检测到,从而表明生长显著或快速。组织病理学特征为弥漫性生长模式以及多形性肿瘤细胞胞质清晰且丰富,其大小和核形态因患者而异。凋亡比有丝分裂更常见。纤维化和局灶性坏死常见,3例有硬化。我们认为B细胞型原发性纵隔淋巴瘤是一种新型的B淋巴瘤变体。

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