Ghez David, Damotte Diane, Perrine Susan P, Faller Douglas V, Canioni Danièle, Brousse Nicole, Lefrère François, Varet Bruno, Hermine Olivier
Service d'Hematologie, Hopital Necker, Paris, France.
Clin Lymphoma Myeloma. 2006 Mar;6(5):417-9. doi: 10.3816/CLM.2006.n.022.
Natural killer (NK) cell malignancies have been associated with neutropenia and disturbances of liver function tests, thought to be related to high levels of soluble Fas ligand (FasL) in the circulation. We report a case of fulminant hepatitis occurring 3 weeks after the initiation of salvage therapy by arginine butyrate and ganciclovir for refractory Epstein-Barr virus-positive NK cell lymphoma. Pathologic examination revealed disappearance of the NK tumor and massive liver injury caused by apoptosis of virtually all hepatocytes. Immunohistochemistry revealed an intense staining for FasL. To our knowledge, this is the first description of the occurrence of FasL-mediated lethal hepatitis after lysis of a NK cell lymphoma.
自然杀伤（NK）细胞恶性肿瘤与中性粒细胞减少及肝功能检查异常有关，据认为这与循环中高水平的可溶性Fas配体（FasL）有关。我们报告1例难治性爱泼斯坦-巴尔病毒阳性NK细胞淋巴瘤患者，在接受精氨酸丁酸盐和更昔洛韦挽救治疗3周后发生暴发性肝炎。病理检查显示NK肿瘤消失，几乎所有肝细胞凋亡导致严重肝损伤。免疫组织化学显示FasL染色强烈。据我们所知，这是首次描述NK细胞淋巴瘤溶解后发生FasL介导的致死性肝炎。
