中国麻风皮肤病杂志 ›› 2024, Vol. 40 ›› Issue (11): 781-784.doi: 10.12144/zgmfskin202411781

• 临床研究 • 上一篇    下一篇

淋巴细胞变异型嗜酸性粒细胞增多症一例并文献复习

居瑞雪   

  1. 潍坊市人民医院检验科，山东潍坊，261041
  • 出版日期:2024-11-15 发布日期:2024-10-14

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

JU Ruixue   

  1. Department of Clinical Laboratory Examination, Weifang People's Hospital, Weifang 261041, China
  • Online:2024-11-15 Published:2024-10-14

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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。

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

Abstract: 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.

Key words: rash, lymphadenopathy, hypereosinophilic syndrome, lymphocytic variant of hypereosinophilic syndrome