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获得性血友病 A 继发于多发性骨髓瘤:机械二尖瓣患者的治疗。

Acquired haemophilia a secondary to multiple myeloma: management of a patient with a mechanical mitral valve.

机构信息

Anesthesiology, NewYork-Presbyterian Hospital/Weill Cornell Medical Center, New York, NY, USA

Hematology/Oncology, George Washington University Medical Faculty Associates, Washington, DC, USA.

出版信息

BMJ Case Rep. 2020 Sep 6;13(9):e230798. doi: 10.1136/bcr-2019-230798.

Abstract

A 77-year-old man with a mechanical mitral valve on warfarin presented with an acute drop in haemoglobin and large spontaneous haematoma. He was found to have a new coagulopathy with initial labs notable for a prolonged activated partial thromboplastin time (APTT). Further workup revealed factor VIII levels less than 1%, abnormal mixing studies and elevated Bethesda titres, which was consistent with an acquired factor VIII inhibitor. Given his bone marrow biopsy result, which was positive for plasma cell myeloma, this coagulopathy was thought to be an acquired haemophilia A secondary to multiple myeloma. Anticoagulation was a challenge in this patient given his mechanical mitral valve and acquired haemophilia A. Although the patient was at risk of thrombosis due to a mechanical mitral valve, he had a bleeding diathesis and anaemia not responsive to transfusion. The decision was made to hold anticoagulation and the patient was started on myeloma treatment which included CyBorD, rituximab and daratumumab. After initiation of treatment APTT and factor VIII normalised. He eventually restarted anticoagulation under direction of his primary care doctor.

摘要

一位 77 岁男性,因机械二尖瓣换瓣服用华法林,出现血红蛋白急性下降和大自发性血肿。他被发现存在新的凝血功能障碍,最初的实验室检查表现为延长的激活部分凝血活酶时间(APTT)。进一步检查显示因子 VIII 水平低于 1%,混合功能异常和贝塞斯达效价升高,与获得性 VIII 因子抑制剂相符。鉴于骨髓活检结果为浆细胞骨髓瘤阳性,考虑该凝血功能障碍为多发性骨髓瘤继发的获得性血友病 A。鉴于该患者存在机械二尖瓣和获得性血友病 A,抗凝治疗极具挑战。虽然该患者因机械二尖瓣存在血栓形成风险,但他有出血倾向和贫血,输血无效。决定停止抗凝治疗,并开始对患者进行骨髓瘤治疗,包括 CyBorD、利妥昔单抗和达雷妥尤单抗。治疗开始后,APTT 和因子 VIII 恢复正常。最终,在他的初级保健医生的指导下,他重新开始抗凝治疗。

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