血管免疫母细胞性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 infiltration of lymph nodes by neoplastic cells of T follicular helper cell origin with a polymorphous inflammatory background including markedly increased follicular dendritic cells and EBV-positive B-cells, as well as prominent proliferation of high endothelial venules. The spleen, liver, skin, and bone marrow are also frequently involved. Patients typically present with generalized lymphadenopathy, hepatosplenomegaly, systemic symptoms, and polyclonal hypergammaglobulinemia. Pruritic skin rash, arthritis, pleural effusion, and ascites may also be observed. The condition is aggressive with generally poor prognosis.

别名

免疫母细胞性淋巴结病

基本事实

遗传方式

不适用

发病年龄

成年期

患病率

<1 / 1 000 000（United States）

外部标识与链接