希培耳—林顿氏病

希培耳—林顿氏病是一种播散性皮肤和内脏毛细血管瘤病。常有囊肿形成。肿瘤位于脑脊液内被称之为毛细血管性血管母细胞瘤(hemangioblastomas),该病变最常见于小脑(36～60％);见于脑干和脊髓者低于5％。视网膜肿瘤亦为其特征性改变(】50％)。血管母细胞瘤罕见于大脑半球。合并内脏病变者包括肾细胞癌(25～38％)和嗜铬细胞瘤(】10％)。该病为常染色体显性遗传,遗传特性外显率近于100％。在性别方面无差异。

Hairy cell leukemia presenting as multiple discrete hepatic lesions

The involvement of hairy cell leukemia in the liver is in the form of portal and sinusoidal cellular infiltration. Here we describe the first case of hepatic hairy cell leukemia presenting as multiple discrete lesions,which was treated successfully.We suggest that in the inves- tigation of discrete hepatic lesions in cases of cancer of unknown primary,hairy cell leukemia should be consid- ered.The excellent response of hairy cell leukemia to therapy highlights the need for such a consideration.