获得性血友病A（FVIII缺乏症）与甲状腺乳头状癌相关：用重组猪FVIII治疗。

Acquired Hemophilia A (FVIII Deficiency) Associated with Papillary Thyroid Cancer: Treatment with Recombinant Porcine FVIII.

作者信息

Nguyen S, Teh P, Zhou J, Chang E Y, von Drygalski A

机构信息

Department of Medicine, University of California San Diego, San Diego, CA, USA.

Department of Medicine, University of California Riverside, Riverside, CA, USA.

出版信息

Case Rep Hematol. 2019 Aug 28;2019:9026121. doi: 10.1155/2019/9026121. eCollection 2019.

DOI:10.1155/2019/9026121

PMID:31559096

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6735189/

Abstract

Acquired hemophilia A (AHA) is a rare autoimmune disorder caused by autoantibodies against Factor VIII (FVIII). It has a high mortality due to bleeding complications. FVIIa-based bypassing agents are the first line of treatment but not always effective. Recombinant porcine (rp) FVIII (Obizur®) was recently approved for rescue treatment but with little evidence-based information regarding efficacy. We report a case of papillary thyroid cancer associated with AHA malignancy that responded to a single dose of rpFVIII after failure to achieve hemostasis with FVIIa-based bypassing products.

摘要

获得性血友病A（AHA）是一种由抗凝血因子VIII（FVIII）自身抗体引起的罕见自身免疫性疾病。由于出血并发症，其死亡率很高。基于FVIIa的旁路制剂是一线治疗药物，但并非总是有效。重组猪（rp）FVIII（Obizur®）最近被批准用于挽救治疗，但关于其疗效的循证信息很少。我们报告了1例与AHA相关的甲状腺乳头状癌病例，在使用基于FVIIa的旁路产品未能实现止血后，单剂量rpFVIII治疗有效。