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因先天性肝内胆管缺如综合征导致肝功能障碍的霍奇金淋巴瘤患者接受 Brentuximab vedotin 治疗后获得部分缓解,且无严重不良事件。

Administration of brentuximab vedotin to a Hodgkin lymphoma patient with liver dysfunction due to vanishing bile duct syndrome resulting in a partial response without any severe adverse events.

机构信息

Department of Hematology, University of Tsukuba Hospital, Tsukuba, Japan.

Department of Hematology, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan.

出版信息

J Clin Exp Hematop. 2022 Sep 28;62(3):154-157. doi: 10.3960/jslrt.21035. Epub 2022 Jul 12.

Abstract

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.

摘要

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。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ebd/9635035/3401576a7ad7/jslrt-62-154-g001.jpg

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