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伴巨大淋巴结病的窦性组织细胞增多症。对患者登记处发生的14例死亡病例的分析。

Sinus histiocytosis with massive lymphadenopathy. An analysis of 14 deaths occurring in a patient registry.

作者信息

Foucar E, Rosai J, Dorfman R F

出版信息

Cancer. 1984 Nov 1;54(9):1834-40. doi: 10.1002/1097-0142(19841101)54:9<1834::aid-cncr2820540911>3.0.co;2-f.

DOI:10.1002/1097-0142(19841101)54:9<1834::aid-cncr2820540911>3.0.co;2-f
PMID:6478418
Abstract

Sinus histiocytosis with massive lymphadenopathy (SHML) is generally regarded as a benign disorder in spite of its propensity to form large masses and to disseminate to both nodal and extranodal sites. Although in most patients the extent of SHML does not appear to determine disease outcome, recent reports have documented that infiltrates of SHML can cause death. To gain perspective on the role of SHML in patient deaths in this population, the authors analyzed the 14 known fatalities in an SHML registry comprising 215 patients. The average age at death was 33 years. Autopsy was performed on eight patients. SHML infiltrates were clearly the cause of death in only two patients (previously reported). In another four cases persistent SHML was prominent at death. The other deaths were the result of complications of defined immunologic abnormalities (five), or of unusual infections (three). The authors have not seen an example of cytologic malignant transformation of SHML.

摘要

伴巨大淋巴结病的窦性组织细胞增生症(SHML)尽管易于形成巨大肿块并扩散至淋巴结和结外部位,但通常被视为一种良性疾病。虽然在大多数患者中,SHML的范围似乎并不决定疾病的结局,但最近的报告表明,SHML浸润可导致死亡。为了了解SHML在该人群患者死亡中的作用,作者分析了一个包含215例患者的SHML登记册中的14例已知死亡病例。平均死亡年龄为33岁。对8例患者进行了尸检。SHML浸润显然仅在2例患者中是死亡原因(先前已报道)。在另外4例病例中,持续性SHML在死亡时很突出。其他死亡是明确的免疫异常并发症(5例)或不寻常感染(3例)的结果。作者尚未见过SHML发生细胞学恶性转化的例子。

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