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皮下脂膜炎样T细胞淋巴瘤

Subcutaneous Panniculitis-Like T-cell Lymphoma

作者信息

Musick Sierra R., Lynch David T.

机构信息

SAMMC

PMID:30860752
Abstract

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma composed of cytotoxic alpha-beta T-cells that mimics panniculitis. Distinction from the more aggressive primary cutaneous gamma delta T-cell lymphoma was made in the 2008 revision of the World Health Organization (WHO) Classification of Tumours of Hematopoietic and Lymphoid Tissues. Autoimmune diseases occur in approximately 20% of cases, and lupus erythematosus panniculitis (LEP) is usually part of the differential diagnosis due to similar clinical and histologic features. Histologically, the neoplastic CD8+, beta F1 expressing cytotoxic T-cells characteristically surround and disrupt individual adipocyte membranes (see Subcutaneous Panniculitis). Most cases have a good prognosis and follow an indolent clinical course; however, 15% to 20% of cases are complicated by hemophagocytic syndrome (HPS).

摘要

皮下脂膜炎样T细胞淋巴瘤(SPTCL)是一种罕见的原发性皮肤淋巴瘤,由细胞毒性α-βT细胞组成,可模仿脂膜炎。在2008年世界卫生组织(WHO)造血和淋巴组织肿瘤分类修订版中,它与侵袭性更强的原发性皮肤γδT细胞淋巴瘤得以区分。自身免疫性疾病约在20%的病例中出现,由于临床和组织学特征相似,狼疮性脂膜炎(LEP)通常是鉴别诊断的一部分。组织学上,肿瘤性CD8 +、表达βF1的细胞毒性T细胞特征性地围绕并破坏单个脂肪细胞膜(见皮下脂膜炎)。大多数病例预后良好,临床病程呈惰性;然而,15%至20%的病例会并发噬血细胞综合征(HPS)。

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