Radiation Oncology, Queensland Health, Brisbane, Queensland, Australia
Haematology, Queensland Health, Brisbane, Queensland, Australia.
BMJ Case Rep. 2024 Sep 24;17(9):e261879. doi: 10.1136/bcr-2024-261879.
Hodgkin's lymphoma (HL)-associated vanishing bile duct syndrome (VBDS) is a paraneoplastic phenomenon leading to cholestasis, end-stage liver failure and potentially death, due to cholestatic liver dysfunction typically precluding the commencement of curative intent chemotherapy. A female in her 20s presented with pruritus, jaundice and cholestatic hepatitis on laboratory tests, confirmed as VBDS on liver biopsy. CT of the chest demonstrated a mediastinal mass and widespread cervical lymphadenopathy. The patient received 30.6 Gy in 17 fractions to the involved sites of disease which led to a marked improvement in liver function, allowing curative intent chemotherapy to be initiated. The patient achieved complete metabolic response and at the most recent follow-up she had no signs of recurrent disease and near-normal liver function tests. This demonstrates that tumour-directed radiotherapy can be used as a potential bridge to curative chemotherapy in early stage HL-associated VBDS.
霍奇金淋巴瘤(HL)相关的进行性肝内胆管消失综合征(VBDS)是一种副肿瘤现象,可导致胆汁淤积、终末期肝功能衰竭,甚至死亡,因为胆汁淤积性肝功能障碍通常排除了有治愈意图的化疗的开始。一名 20 多岁的女性出现瘙痒、黄疸和胆汁淤积性肝炎的实验室检查结果,肝活检证实为 VBDS。胸部 CT 显示纵隔肿块和广泛的颈部淋巴结病。患者接受了累及疾病部位的 30.6Gy 共 17 次分割放疗,导致肝功能显著改善,从而能够开始有治愈意图的化疗。患者达到了完全代谢缓解,在最近的随访中,她没有疾病复发的迹象,肝功能检查接近正常。这表明肿瘤定向放疗可作为早期 HL 相关 VBDS 向有治愈意图的化疗的潜在桥梁。