肝脾T细胞淋巴瘤

定义 英文原文（暂无中文）

A rare T-cell non-Hodgkin lymphoma characterized by a proliferation of cytotoxic T-cells, usually gamma delta T-cells, with involvement of the liver and spleen, but without involvement of lymph nodes. The bone marrow is consistently affected. Patients typically present during adolescence or young adulthood with hepatosplenomegaly, pancytopenia, and systemic symptoms. Peripheral blood involvement may develop later in the disease course. There is a clear male preponderance. The disease often occurs in the context of long-term immunosuppression. The course is aggressive with poor therapy response.

基本事实

遗传方式

不适用

发病年龄

青少年期、成年期

患病率

<1 / 1 000 000（United States）

外部标识与链接