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

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.