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恶性组织细胞增多症(组织细胞性髓性网状细胞增多症)。I. 29例临床病理研究

Malignant histiocytosis (histiocytic medullary reticulosis). I. Clinicopatholigic study of 29 cases.

作者信息

Warnke R A, Kim H, Dorfman R F

出版信息

Cancer. 1975 Jan;35(1):215-30. doi: 10.1002/1097-0142(197501)35:1<215::aid-cncr2820350127>3.0.co;2-h.

DOI:10.1002/1097-0142(197501)35:1<215::aid-cncr2820350127>3.0.co;2-h
PMID:1167345
Abstract

The clinical records and histologic material from 29 cases of malignant histiocytosis (MH) have been reviewed, as well as autopsy findings in 14 cases. The mean age was 31 years, with a 2.2:1 male to female preponderance. Major physical findings included temperature elevation, lymphadenopathy, hepatomegaly, splenomegaly, and preterminal jaundice. Common laboratory findings were anemia, leukopenia, and thrombocytopenia. The median survival was 6 months, the mean 14 months, and the range from 1 month to 8 years. The histologic features observed in lymph nodes, liver, spleen, and bone marrow have been emphasized, as well as features useful in differential diagnosis. In contrast to our experience with the non-Hodgkin's lymphomas, bone marrow aspiration was superior to biopsy in assessing marrow involvement. Unusual manifestations included soft tissue infiltration in 5 cases; 2 of these patients presented with a soft tissue mass. The distinctive clinical as well as histologic findings warrant recognition and separation of MH from other hematopoietic disorders.

摘要

回顾了29例恶性组织细胞增多症(MH)的临床记录和组织学材料,以及14例尸检结果。平均年龄为31岁,男女比例为2.2:1。主要体格检查发现包括体温升高、淋巴结病、肝肿大、脾肿大和临终前黄疸。常见实验室检查结果为贫血、白细胞减少和血小板减少。中位生存期为6个月,平均为14个月,范围为1个月至8年。强调了在淋巴结、肝脏、脾脏和骨髓中观察到的组织学特征以及对鉴别诊断有用的特征。与我们在非霍奇金淋巴瘤方面的经验相比,骨髓穿刺在评估骨髓受累方面优于活检。不寻常的表现包括5例软组织浸润;其中2例患者表现为软组织肿块。独特的临床和组织学发现值得将MH与其他造血系统疾病区分开来并加以识别。

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