Division of Hematopathology, Mayo Clinic, Rochester, MN, United States of America.
Division of Hematology, Mayo Clinic, Rochester, MN, United States of America.
Ann Diagn Pathol. 2020 Feb;44:151412. doi: 10.1016/j.anndiagpath.2019.151412. Epub 2019 Oct 18.
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.
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。