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两名儿童发生的伴有噬血细胞现象的侵袭性皮下脂膜炎样T细胞淋巴瘤(皮下脂膜炎样T细胞淋巴瘤)

Aggressive subcutaneous panniculitis-like T-cell lymphoma with hemophagocytosis in two children (subcutaneous panniculitis-like T-cell lymphoma).

作者信息

Koh Mark Jean-Aan, Sadarangani Sapna P, Chan Yuin-Chew, Chan Mei-Yoke, Tan Ah-Moy, Tan Suat-Hoon, Tay Yong-Kwang, Ng Siok-Bian

机构信息

Department of Dermatology, Changi General Hospital, Singapore.

出版信息

J Am Acad Dermatol. 2009 Nov;61(5):875-81. doi: 10.1016/j.jaad.2009.01.045. Epub 2009 Sep 9.

Abstract

Subcutaneous panniculitis-like T-cell lymphoma is an uncommon form of cutaneous lymphoma in the pediatric population. It is characterized histologically by subcutaneous infiltration of pleomorphic cytotoxic T cells, mimicking a lobular panniculitis. Although usually described as having an indolent clinical course, the condition may be complicated by systemic involvement and hemophagocytic syndrome, resulting in a poorer prognosis. We present two pediatric patients with aggressive forms of subcutaneous panniculitis-like T-cell lymphoma complicated by hemophagocytic syndrome, and discuss the current literature.

摘要

皮下脂膜炎样T细胞淋巴瘤是儿科人群中一种罕见的皮肤淋巴瘤形式。其组织学特征为多形性细胞毒性T细胞浸润皮下,类似小叶性脂膜炎。尽管通常被描述为具有惰性临床病程,但该疾病可能并发全身受累和噬血细胞综合征,导致预后较差。我们报告了两名患有侵袭性皮下脂膜炎样T细胞淋巴瘤并并发噬血细胞综合征的儿科患者,并讨论了当前的文献。

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