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累及皮下组织并伴有噬血细胞综合征的γ/δ T细胞淋巴瘤。

Gamma/delta T-cell lymphoma involving the subcutaneous tissue and associated with a hemophagocytic syndrome.

作者信息

Avinoach I, Halevy S, Argov S, Sacks M

机构信息

Dermatopathology Unit, Faculty of Health Sciences, Soroka University Medical Center, Ben-Gurion University of the Negev, Beer-Sheva, Israel.

出版信息

Am J Dermatopathol. 1994 Aug;16(4):426-33. doi: 10.1097/00000372-199408000-00014.

DOI:10.1097/00000372-199408000-00014
PMID:7978074
Abstract

A case of gamma/delta-positive T-cell lymphoma localized primarily in the subcutaneous adipose tissue and associated with an extensive hemophagocytic syndrome and a rapid, fatal outcome is presented. Previous reports of gamma/delta cutaneous and subcutaneous T-cell lymphomas are reviewed. To define the characteristics of a T-cell subset of neoplastic cells bearing the gamma/delta receptor in a subcutaneous lymphoma, we undertook clinical and laboratory examinations, morphological evaluation of biopsies, electron microscopy, and extensive immunohistochemical studies. Immunohistochemical studies showed that the neoplastic cells expressed the gamma/delta T-cell receptor (CD3+, C gamma M1+) and were CD2+, CD43+, CD45+, CD45RO+, PCNA+, but were beta F1-, CD1-, CD4-, CD8-, CD15-, CD20-, CD25-, CD30-, CD45R-, CD57-, CD68-, Mac 387-, and HLA Dr-. To the best of our knowledge, this is the first report of a subcutaneous T-cell lymphoma of gamma/delta type involving the subcutaneous tissue and associated with a hemophagocytic syndrome.

摘要

本文报告了一例主要局限于皮下脂肪组织的γ/δ阳性T细胞淋巴瘤,该病例伴有广泛的噬血细胞综合征且预后迅速、致命。回顾了既往关于γ/δ皮肤和皮下T细胞淋巴瘤的报道。为明确皮下淋巴瘤中携带γ/δ受体的肿瘤细胞T细胞亚群的特征,我们进行了临床和实验室检查、活检标本的形态学评估、电子显微镜检查以及广泛的免疫组织化学研究。免疫组织化学研究显示,肿瘤细胞表达γ/δ T细胞受体(CD3+、CγM1+),且CD2+、CD43+、CD45+、CD45RO+、PCNA+,但βF1-、CD1-、CD4-、CD8-、CD15-、CD20-、CD25-、CD30-、CD45R-、CD57-、CD68-、Mac 387-及HLA Dr-。据我们所知,这是首例累及皮下组织并伴有噬血细胞综合征的γ/δ型皮下T细胞淋巴瘤报告。

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Primary cutaneous γ/δ T-cell lymphoma. An atypical case with bone marrow granulomas.原发性皮肤γ/δ T细胞淋巴瘤。一例伴有骨髓肉芽肿的非典型病例。
J Dermatol Case Rep. 2015 Mar 31;9(1):15-8. doi: 10.3315/jdcr.2015.1191.
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Primary cutaneous T-cell lymphoma of unspecified type with cytotoxic phenotype: clinicopathological analysis of 27 patients.
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Cancer Sci. 2009 Jan;100(1):33-41. doi: 10.1111/j.1349-7006.2008.01000.x. Epub 2008 Oct 23.
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Hepatosplenic and subcutaneous panniculitis-like gamma/delta T cell lymphomas are derived from different Vdelta subsets of gamma/delta T lymphocytes.肝脾和皮下脂膜炎样γ/δ T细胞淋巴瘤源自γ/δ T淋巴细胞的不同Vδ亚群。
J Mol Diagn. 2000 Feb;2(1):11-9. doi: 10.1016/s1525-1578(10)60610-1.