Department of Haematology, Faculty of Medicine, School of Health Sciences, University of Ioannina, Ioannina, Greece.
Department of Internal Medicine, Faculty of Medicine, School of Health Sciences, University of Ioannina, Ioannina, Greece.
In Vivo. 2021 Jul-Aug;35(4):1951-1957. doi: 10.21873/invivo.12462.
Hepatic dysfunction in patients with classical Hodgkin lymphoma (cHL) is of multifactorial aetiology. Prompt evaluation with laboratory tests and imaging methods is sufficient for diagnosis in most cases. Intrahepatic cholestasis and vanishing bile duct syndrome (VBDS) may complicate cHL as rare paraneoplastic phenomena. Liver biopsy provides crucial evidence of cholestasis, and ductopenia, if present, confirms the diagnosis of VBDS.
We report on a cHL patient that presented with jaundice and bulky mediastinal disease and unfold the therapeutic dilemmas we confronted. Marked hyperbilirubinemia was successfully reversed with brentuximab vedotin (BV) at a dose of 1.2 mg/kg and the patient was subsequently treated with doxorubicin, bleomycin, vinblastine and dacarbazine (ABVD) at full doses, achieving complete metabolic response. A literature review of intrahepatic cholestasis in cHL is also presented based on currently available data with focus on treatment options and clinicopathologic associations.
VBDS and intrahepatic cholestasis are rare and potentially fatal complications of cHL. Their prompt recognition and appropriate treatment can dramatically affect cHL patients' outcome. BV, used at a reduced dose as a bridging therapy, should be considered as a high-priority treatment plan in these challenging cases.
经典霍奇金淋巴瘤（cHL）患者的肝功能障碍具有多因素病因。在大多数情况下，通过实验室检查和影像学方法进行快速评估即可确诊。肝内胆汁淤积和消失胆管综合征（VBDS）可能作为罕见的副肿瘤现象使 cHL 复杂化。肝活检提供了胆汁淤积和如果存在的胆管减少的关键证据，从而确诊 VBDS。
我们报告了一例以黄疸和巨大纵隔疾病为表现的 cHL 患者，并阐述了我们所面临的治疗困境。用 1.2mg/kg 剂量的 Brentuximab vedotin（BV）成功逆转了显著的高胆红素血症，随后患者接受了多柔比星、博来霉素、长春碱和达卡巴嗪（ABVD）的全剂量治疗，达到了完全代谢缓解。根据现有数据，我们还对 cHL 中的肝内胆汁淤积进行了文献回顾，重点介绍了治疗选择和临床病理关联。
VBDS 和肝内胆汁淤积是 cHL 的罕见且潜在致命的并发症。它们的快速识别和适当治疗可以显著影响 cHL 患者的预后。作为桥接治疗，以较低剂量使用的 BV 应被视为这些具有挑战性病例的高度优先治疗方案。
