淋巴细胞变异型嗜酸性粒细胞增多症一例并文献复习  

Lymphocytic variant of hypereosinophilic syndrome:a case report and literature review

作　　者：居瑞雪[1] JU Ruixue(Department of Clinical Laboratory Examination,Weifang People's Hospital,Weifang 261041,China)

机构地区：[1]潍坊市人民医院检验科,山东潍坊261041

出　　处：《中国麻风皮肤病杂志》2024年第11期781-784,共4页China Journal of Leprosy and Skin Diseases

基　　金：潍坊市卫生健康委员会项目(编号:wfwsjk-2019-001)。

摘　　要：淋巴细胞变异型嗜酸性粒细胞增多症(lymphocytic variant of hypereosinophilic syndrome,L-HES)是一种异常的克隆T淋巴细胞引起的嗜酸性粒细胞增多症,其特征为骨髓或外周血中存在表型异常的T淋巴细胞群。本文报道一例49岁L-HES男性患者,嗜酸性粒细胞增多症病史13年,半年前出现全身多处淋巴结肿大,病理示皮病性淋巴结炎,伴反应性增生及嗜酸性粒细胞浸润。骨髓流式细胞术检测示嗜酸性粒细胞比例增高(19.3%),异常T淋巴细胞群,免疫表型为CD3(-)CD4(+)CD8(-)CD5brightCD7(-)。给予口服甲泼尼龙36 mg/d好转,出院后甲泼尼龙减量至8 mg/d维持治疗可控制病情。Lymphocytic variant of hypereosinophilic syndrome(L-HES)is a kind of hypereosinophilia caused by abnormal clonal T lymphocytes.It is characterized by the presence of phenotypically abnormal T lymphocyte populations in bone marrow or peripheral blood.Herein,we report a 49-year-old man with a 13-year history of hypereosinophilia.The multiple lymph nodes were enlarged since half a year ago,and the histopathological examination showed dermatopathic lymphadenitis of lymph nodes with reactive hyperplasia and eosinophilic infiltration.Bone marrow flow cytometry detected a group of abnormal T lymphocytes with CD3(-)CD4(+)CD8(-)CD5brightCD7(-).Oral methylprednisolone was effective(36 mg/d).Methylprednisolone dose was reduced to 8 mg/d after discharge and the disease is well controlled.

关 键 词：皮疹 淋巴结肿大 嗜酸性粒细胞增多症 淋巴细胞变异型嗜酸性粒细胞增多症 

分 类 号：R557.5[医药卫生—血液循环系统疾病]