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一种表现为狮面的罕见皮肤T细胞淋巴瘤。

An unusual cutaneous T cell lymphoma presenting as leonine facies.

作者信息

Vocks E, Hertenberger B, Worret W I, Kaudewitz P, Fellbaum C, Ring J

机构信息

Department of Dermatology and Allergology Biederstein, Technical University of Munich, Munich, Germany.

出版信息

Eur J Dermatol. 2000 Jun;10(4):309-12.

Abstract

We report an unusual case of a cutaneous T cell lymphoma with the clinical picture of leonine facies as the only skin symptom appearing during the first years of the disease. Small atypical lymphocytes with partly pleomorphic, partly indented cerebriform nuclei are present in the facial skin as well as in the peripheral blood and in the bone marrow. The lymphoma shows an indolent clinical behaviour without rapid progression of the disease. It shares features with both pleomorphic small/medium-sized T cell lymphoma and mycosis fungoides, but cannot be classified according to the criteria of the European Organization for Research and Treatment of Cancer (EORTC) Classification for Primary Cutaneous Lymphomas. At present the lymphoma has to be ranged among the group of so-called unspecified peripheral T cell lymphomas according to the proposal of the International Lymphoma Study Group.

摘要

我们报告了一例罕见的皮肤T细胞淋巴瘤病例,在疾病的最初几年,其临床表现为狮面样面容,这是唯一出现的皮肤症状。面部皮肤、外周血和骨髓中均存在具有部分多形性、部分凹陷脑回状核的小非典型淋巴细胞。该淋巴瘤临床行为惰性,疾病无快速进展。它兼具多形性小/中型T细胞淋巴瘤和蕈样肉芽肿的特征,但无法根据欧洲癌症研究与治疗组织(EORTC)原发性皮肤淋巴瘤分类标准进行分类。根据国际淋巴瘤研究组的提议,目前该淋巴瘤必须归类于所谓的未特定外周T细胞淋巴瘤组。

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