Cameron Andrew M, Truty Jadwiga, Truell Jeff, Lassman Charles, Zimmerman Michael A, Kelly Burnett S, Farmer Douglas G, Hiatt Jonathan R, Ghobrial Rafik, Busuttil Ronald W
Division of Liver and Pancreas Transplantation, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095-7054, USA.
Transplantation. 2005 Oct 15;80(7):993-6. doi: 10.1097/01.tp.0000173999.09381.95.
Systemic lymphomas may involve the liver but rarely cause fulminant hepatic failure (FHF). Acute liver failure from primary hepatic lymphoma (PHL) is even less common with most patients succumbing to the sequelae of FHF before the correct diagnosis is made. We report a patient who underwent successful orthotopic liver transplant (OLT) and chemotherapy for FHF secondary to PHL. This previously-well male developed profound coagulopathy and encephalopathy 6 weeks after the onset of jaundice and fatigue. Workup failed to reveal the underlying cause of his liver failure and the patient soon required urgent OLT. Pathologic evaluation of his explanted liver revealed a malignant T-cell rich, large B-cell non-Hodgkin's lymphoma with widespread hepatocellular necrosis. The patient made an excellent clinical recovery and is undergoing CHOP-Rituxan chemotherapy. This scenario demonstrates that lymphoma should be considered in the differential diagnosis of FHF without clear etiology because of the potential for intervention with transplant and chemotherapy.
系统性淋巴瘤可能累及肝脏，但很少导致暴发性肝衰竭（FHF）。原发性肝淋巴瘤（PHL）导致的急性肝衰竭更为罕见，大多数患者在做出正确诊断之前就死于FHF的后遗症。我们报告了一例因PHL继发FHF而接受原位肝移植（OLT）和化疗且获得成功的患者。这位既往健康的男性在出现黄疸和疲劳6周后出现严重凝血功能障碍和脑病。检查未能揭示其肝衰竭的潜在病因，患者很快就需要紧急OLT。对其切除肝脏的病理评估显示为富含恶性T细胞的大B细胞非霍奇金淋巴瘤，并伴有广泛的肝细胞坏死。患者临床恢复良好，正在接受CHOP-利妥昔单抗化疗。这种情况表明，对于病因不明的FHF，在鉴别诊断中应考虑淋巴瘤，因为有可能通过移植和化疗进行干预。
