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Primary Cutaneous T-Cell Lymphomas Showing Gamma-Delta (γδ) Phenotype and Predominantly Epidermotropic Pattern are Clinicopathologically Distinct From Classic Primary Cutaneous γδ T-Cell Lymphomas.

具有 γδ T细胞表型和明显亲表皮模式的皮肤原发T细胞淋巴瘤临床病理学特点不同于经典的皮肤原发 γδ T细胞淋巴瘤

Merrill ED,Agbay R,Miranda RN,Aung PP,Tetzlaff MT,Young KH,Curry JL,Nagarajan P,Ivan D,Prieto VG,Medeiros LJ,Duvic M,Torres-Cabala CA

Abstract

Primary cutaneous gamma-delta (γδ) T-cell lymphoma is a rare disease that typically involves the dermis and subcutis. Cases of primary cutaneous T-cell lymphomas showing γδ phenotype and predominantly epidermotropic pattern (EγδTCL) are not well defined. In this series, cases of primary cutaneous T-cell lymphomas showing γδ phenotype were reviewed and classified as predominantly epidermotropic (EγδTCL) when >75% of lymphoma cells resided in the epidermis or predominantly dermal and/or subcutaneous (DSγδTCL). Clinical, pathologic, and immunophenotypic features were compared in 27 biopsies from 13 patients of EγδTCL and 13 biopsies from 7 patients of DSγδTCL. The lymphoma cells were diffusely positive for CD3 and T-cell receptor (TCR)γ, mostly positive for granzyme B and TIA-1, variably positive for CD8, CD7, and CD30, and negative for CD4 and TCRβ. Two patients with EγδTCL had dissemination to lymph nodes and 1 to the lung; 1 patient with DSγδTCL had gastrointestinal involvement. The median survival of patients with EγδTCL was not reached, and with a median follow-up of 19.2 months, 3/13 died. In contrast, the median survival of patients with DSγδTCL was 10 months, and after a median follow-up of 15.6 months, 5/5 died (P<0.01). EγδTCL is a rare presentation of cutaneous T-cell lymphoma that can be distinguished from DSγδTCL based on the extent of epidermotropism and has a better prognosis and longer median survival than DSγδTCL. However, although EγδTCL resembles mycosis fungoides clinically and histologically, a subset of EγδTCL is more likely to behave more aggressively than typical mycosis fungoides.

摘要

原发于皮肤的γδT细胞淋巴瘤是一种罕见的疾病,通常累及真皮和皮下组织。原发于皮肤的、具有 γδT细胞表型和明显亲表皮模式的T细胞淋巴瘤(EγδTCL)还没有很好的定义。

本研究中,重新复习了具有γδT细胞表型的皮肤T细胞淋巴瘤,当75%以上肿瘤细胞位于表皮时归为EγδTCL,75%以上肿瘤细胞位于真皮和/或皮下组织时归为DSγδTCL。分析样本的临床、病理和免疫表型,其中13例EγδTCL患者的27份活检标本,7例DSγδTCL患者的13份活检标本。肿瘤细胞弥漫表达CD3、TCRγ,大多数表达 granzyme B 和 TIA-1, 不同程度表达 CD8, CD7,和 CD30,不表达CD4 和TCRβ。2例EγδTCL患者播散至淋巴结,1例播散到肺;1例DSγδTCL患者累及胃肠道。EγδTCL患者未达中位生存时间,随访中位时间19.2月,3/13死亡。DSγδTCL患者的中位存活时间10个月,中位随访时间15.6月,原文5/5死亡。EγδTCL是一种罕见的皮肤T细胞淋巴瘤,通过评估表皮侵犯的程度可以将其与DSγδTCL区分开,前者具有较好的预后和更长的中位生存时间。然而,尽管EγδTCL在临床和病理上都类似于蕈样霉菌病,但是其往往比典型的蕈样霉菌病更具有侵袭性。

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