Maggi U, Russo R, Conte G, Chiumello D, Lunghi G, Maggioni M, Caspani M L, Arnoldi R, Dondossola D, Rossi G
U.O. Chirurgia Generale e Trapianti di Fegato, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milano, Milano, Italy.
Transplant Proc. 2011 May;43(4):1184-6. doi: 10.1016/j.transproceed.2011.01.142.
Varicella is a well-known contagious disease of childhood that can also affect both immunodepressed and immunocompetent adults. The present observations concern a previously healthy adult patient who presented with a fulminant hepatitis evolving in multiorgan failure (MOF), associated with an atypical papulo-ethemateous cutaneous rash without fever. An hepatic biopsy showed massive necrosis. Because of the persistent MOF and severe hemodynamic instability, total hepatectomy was performed as a bridge to urgent liver transplantation (OLT). Despite temporary improvement, the patients condition progressively deteriorated and he died 11 hours after the hepatectomy, i.e. 7 days after admission to the intensive care unit. High viral loads of varicella zoster virus (VZV) and human herpes virus 6 (HHV6) were demonstrated in the blood and in DNA at post mortem examination of the liver, kidneys, lung, and heart. We hypothesize that VZV infection may occasionally occur in immunocompetent patients due to extremely virulent strains that can be rapidly fatal. The clinical influence of simultaneous infection with HHV6 is not clear. Moreover, the role of a previous steroid treatment as a trigger for a temporary immunodepressed state must be considered. The diagnosis of liver disease from VZV should always be clinically suspected in the presence of concurrent atypical skin lesions and a temporarily immunocompromised state. Therapy with acyclovir was ineffective in our patient. Based on the wide spectrum of VZV infections, fulminant MOF in immunocompetent adults must raise the possibility of VZV with simultaneous HHV6 infection with early listing of the patient for a urgent OLT, possibly with a total hepatectomy as a bridge, due to the therapeutic uncertainty of medical treatments.
水痘是一种广为人知的儿童期传染病，也可影响免疫功能低下和免疫功能正常的成年人。本观察涉及一名既往健康的成年患者，该患者出现暴发性肝炎并进展为多器官功能衰竭（MOF），伴有无发热的非典型丘疹性皮疹。肝活检显示大量坏死。由于持续性MOF和严重的血流动力学不稳定，进行了全肝切除术作为紧急肝移植（OLT）的过渡。尽管有短暂改善，但患者病情逐渐恶化，在肝切除术后11小时死亡，即入住重症监护病房7天后死亡。在对肝脏、肾脏、肺和心脏进行尸检时，在血液和DNA中检测到高病毒载量的水痘带状疱疹病毒（VZV）和人疱疹病毒6（HHV6）。我们推测，由于极其毒力的毒株，VZV感染可能偶尔发生在免疫功能正常的患者中，这些毒株可迅速致命。HHV6同时感染的临床影响尚不清楚。此外，必须考虑先前使用类固醇治疗作为暂时免疫抑制状态触发因素的作用。在存在并发非典型皮肤病变和暂时免疫功能低下状态的情况下，应始终临床怀疑VZV引起的肝病诊断。阿昔洛韦治疗对我们的患者无效。基于VZV感染的广泛范围，免疫功能正常的成年人中暴发性MOF必须提高VZV合并HHV6感染的可能性，由于药物治疗的不确定性，应尽早将患者列入紧急OLT名单，可能以全肝切除术作为过渡。
