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皮肤原发性浆细胞瘤

Primary plasmacytoma of the skin.

作者信息

Tüting T, Bork K

机构信息

Department of Dermatology, Johannes-Gutenberg University, Mainz, Germany.

出版信息

J Am Acad Dermatol. 1996 Feb;34(2 Pt 2):386-90. doi: 10.1016/s0190-9622(07)80014-4.

Abstract

Primary plasmacytoma of the skin without evidence of bone marrow plasmacytosis is a rare disorder belonging to the heterogenous spectrum of plasma cell neoplasms. With immunohistochemical techniques, differentiation from benign plasma cell aggregates can be accomplished by demonstrating the monoclonality of tumor cells. We describe a patient in whom a solitary primary cutaneous plasmacytoma developed on the left thigh. Immunohistochemically, plasma cells showed restriction of immunoglobulin lambda-chain expression. Underlying multiple myeloma was excluded by serum protein and immunoglobulin electrophoresis, roentgenographic skeletal survey, and bone marrow biopsy. The tumor responded well to local electron-beam radiotherapy. Whereas multiple primary cutaneous plasmacytomas seem to carry a significant early mortality rate, the prognosis of solitary lesions is likely to be more favorable.

摘要

无骨髓浆细胞增多证据的原发性皮肤浆细胞瘤是一种罕见疾病,属于浆细胞肿瘤的异质性范畴。借助免疫组化技术,通过证明肿瘤细胞的单克隆性可实现与良性浆细胞聚集物的鉴别。我们描述了一名患者,其左大腿出现了孤立性原发性皮肤浆细胞瘤。免疫组化显示,浆细胞呈现免疫球蛋白λ链表达受限。通过血清蛋白和免疫球蛋白电泳、X线骨骼检查以及骨髓活检排除了潜在的多发性骨髓瘤。该肿瘤对局部电子束放疗反应良好。虽然多发性原发性皮肤浆细胞瘤似乎有显著的早期死亡率,但孤立性病变的预后可能更乐观。

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