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高嗜酸性粒细胞综合征患者 T 细胞淋巴增生性疾病的流行率和谱:参考实验室经验。

Prevalence and spectrum of T-cell lymphoproliferative disorders in patients with Hypereosinophilia: A reference laboratory experience.

机构信息

Division of Hematopathology, Mayo Clinic, Rochester, MN, United States of America.

Division of Hematology, Mayo Clinic, Rochester, MN, United States of America.

出版信息

Ann Diagn Pathol. 2020 Feb;44:151412. doi: 10.1016/j.anndiagpath.2019.151412. Epub 2019 Oct 18.

Abstract

Hypereosinophilia (HE) is defined as persistently elevated absolute eosinophil count (AEC) ≥ 1.5 × 10/L, which can be due to a variety of underlying causes. In this study, we investigated the prevalence and spectrum of T-cell lymphoproliferative disorders in 124 consecutive patients with HE by flow cytometric immunophenotyping. Available medical records, pathology reports and T-cell receptor (TCR) gene rearrangement were reviewed. Fifteen patients (12%) with HE had abnormal T-cell populations that were initially detected by flow cytometry. The presence of immunophenotypically abnormal T cells was not associated with higher AEC or higher absolute lymphocyte count levels, in comparison to those without abnormal T cells. Molecular studies concordantly identified a clonal TCR gene rearrangement in 8 of 10 cases tested. Based on the combination of clinical presentation, morphologic findings and laboratory studies, seven patients were diagnosed with the lymphocytic variant of hypereosinophilic syndrome and five with overt T-cell lymphoma (4 peripheral T-cell lymphoma NOS, 1 primary cutaneous T-cell lymphoma). The remaining three had an unknown diagnosis due to lack of information and additional workup would be warranted. These findings underscore the importance of flow cytometry as a screening tool to identify T-cell lymphoproliferative disorders in patients with HE.

摘要

嗜酸性粒细胞增多症(HE)定义为持续升高的绝对嗜酸性粒细胞计数(AEC)≥1.5×10/L,其可能由多种潜在原因引起。在这项研究中,我们通过流式细胞免疫表型分析调查了 124 例连续 HE 患者中 T 细胞淋巴增生性疾病的患病率和谱。回顾了可用的病历、病理报告和 T 细胞受体(TCR)基因重排。15 名(12%)HE 患者的异常 T 细胞群最初通过流式细胞术检测到。与无异常 T 细胞的患者相比,免疫表型异常 T 细胞的存在与更高的 AEC 或更高的绝对淋巴细胞计数水平无关。分子研究一致在 10 例检测病例中的 8 例中鉴定出克隆 TCR 基因重排。基于临床表现、形态学发现和实验室研究的综合,7 名患者被诊断为嗜酸性粒细胞增多综合征的淋巴细胞变体,5 名患者被诊断为显性 T 细胞淋巴瘤(4 例非特指型外周 T 细胞淋巴瘤,1 例原发性皮肤 T 细胞淋巴瘤)。其余 3 名患者由于信息缺乏和需要进一步检查而无法明确诊断。这些发现强调了流式细胞术作为识别 HE 患者 T 细胞淋巴增生性疾病的筛查工具的重要性。

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