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系统性浆细胞增多症。一种具有独特的多发性皮肤疹、全身性淋巴结病和多克隆高球蛋白血症的综合征。

Systemic plasmacytosis. A syndrome of peculiar multiple skin eruptions, generalized lymphadenopathy, and polyclonal hypergammaglobulinemia.

作者信息

Watanabe S, Ohara K, Kukita A, Mori S

出版信息

Arch Dermatol. 1986 Nov;122(11):1314-20. doi: 10.1001/archderm.122.11.1314.

Abstract

A description is given of two patients with peculiar multiple skin eruptions, asymptomatic generalized lymphadenopathy, and polyclonal hypergammaglobulinemia. Both patients were admitted to our hospital for further evaluation of an increased erythrocyte sedimentation rate and hypergammaglobulinemia discovered during routine medical examinations. Despite various investigations, the underlying disease causing the hypergammaglobulinemia was not found. Histologic examination disclosed dense perivascular infiltration of plasma cells in the dermis. In the lymph nodes, considerable plasma cell infiltration was found from the cortex to the medulla. These plasma cells were mature and showed no cellular atypism. The association of peculiar multiple skin eruptions, lymphadenopathy, and polyclonal hypergammaglobulinemia, which we have called "systemic plasmacytosis," signifies a new syndrome that can be differentiated from diseases reported previously.

摘要

本文描述了两名患有特殊多发性皮肤疹、无症状性全身淋巴结病和多克隆高球蛋白血症的患者。两名患者因常规体检发现红细胞沉降率升高和高球蛋白血症而入院作进一步评估。尽管进行了各种检查,但未发现导致高球蛋白血症的潜在疾病。组织学检查显示真皮中有密集的血管周围浆细胞浸润。在淋巴结中,从皮质到髓质均发现大量浆细胞浸润。这些浆细胞成熟,未显示细胞异型性。我们所称的“系统性浆细胞增多症”,即特殊多发性皮肤疹、淋巴结病和多克隆高球蛋白血症的关联,代表了一种可与先前报道的疾病相鉴别的新综合征。

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