Department of Hematology, University of Tsukuba Hospital, Tsukuba, Japan.
Department of Hematology, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan.
J Clin Exp Hematop. 2022 Sep 28;62(3):154-157. doi: 10.3960/jslrt.21035. Epub 2022 Jul 12.
Vanishing bile duct syndrome (VBDS) is a rare hepatic disorder which leads to liver failure as a result of progressive destruction of the intrahepatic bile ducts. There are no treatment modalities for VBDS itself and severe hepatic dysfunction restricts the treatment of underlying diseases. We safely treated a case of classic Hodgkin lymphoma (HL) with VBDS using brentuximab vedotin (BV). The patient was treated with 5 cycles of reduced BV and a partial metabolic response was obtained. Moreover, a standard dose of BV for another 5 cycles was accomplished with minimal adverse events. Our experience indicates that BV could be a treatment option for classic HL with VBDS.
消失胆管综合征(VBDS)是一种罕见的肝脏疾病,可导致肝衰竭,其原因是肝内胆管进行性破坏。目前尚无针对 VBDS 的治疗方法,严重的肝功能障碍限制了基础疾病的治疗。我们使用 Brentuximab vedotin(BV)成功治疗了一例伴有 VBDS 的经典霍奇金淋巴瘤(HL)病例。该患者接受了 5 个周期的减少剂量 BV 治疗,并获得了部分代谢反应。此外,还完成了 5 个周期的标准剂量 BV 治疗,且不良反应最小。我们的经验表明,BV 可能是伴有 VBDS 的经典 HL 的一种治疗选择。