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弥漫性滤泡淋巴瘤变异型,具有典型的诊断模式和异常侵袭性的临床表现。

Diffuse follicular lymphoma variant with a typical diagnostic pattern and an unusually aggressive clinical presentation.

机构信息

Department of Hematology, Ramón y Cajal University Hospital, M-607, km. 9, 100, 28034, Madrid, Spain.

Department of Pathology, Ramón y Cajal University Hospital, Madrid, Spain.

出版信息

Int J Hematol. 2020 Aug;112(2):136-138. doi: 10.1007/s12185-020-02901-1. Epub 2020 Jun 6.

DOI:10.1007/s12185-020-02901-1
PMID:32506320
Abstract

Diffuse follicular lymphoma (FL) variant is a rare condition that shows distinctive clinical, morphological, immunophenotypic, and molecular features that distinguish it from classical FL. Diffuse FL variant is characterized by a predominantly diffuse growth pattern, absence of the t (14;18) IGH/BCL2 translocation, CD23 expression, and presence of 1p36 deletion. Gene mutations involving STAT6 have been reported, with nuclear expression of STAT6 and phosphorylated STAT6 detected by immunohistochemistry. Patients frequently present with inguinal node involvement and low clinical stage. We describe the case of an 80-year-old female diagnosed with diffuse FL variant, presented with a classic diagnostic pattern and an unusual aggressive clinical onset.

摘要

弥漫性滤泡淋巴瘤(FL)变体是一种罕见的疾病,具有独特的临床、形态学、免疫表型和分子特征,与经典 FL 相区别。弥漫性 FL 变体的特征是主要呈弥漫性生长模式,缺乏 t(14;18)IGH/BCL2 易位、CD23 表达和 1p36 缺失。据报道,STAT6 基因的突变涉及其中,免疫组化检测到 STAT6 的核表达和磷酸化 STAT6。患者常表现为腹股沟淋巴结受累和低临床分期。我们描述了一例 80 岁女性被诊断为弥漫性 FL 变体的病例,表现出典型的诊断模式和不寻常的侵袭性临床发病。

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