Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature.
Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature.
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DOI：
10.1155/2017/2057019
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发表时间：
2017
影响因子：
0.7
通讯作者：
Hermans C
中科院分区：
文献类型：
--
作者：
Binet Q;Lambert C;Sacré L;Eeckhoudt S;Hermans C
背景资料。获得性血友病A(AHA)是一种罕见的疾病，是由于自发地形成针对内源性因子的中和抗体所致，约一半的病例与妊娠、产后、自身免疫疾病、恶性肿瘤或药物不良反应有关。症状包括可能危及生命的严重和意想不到的出血。案例研究。我们报告一个与大疱性类天疱疮(BP)相关的AHA病例，这是一种慢性、自身免疫性、真皮下、水疱性皮肤病。据我们所知，这是第25起记录在案的此类关联案件。经过不到3个月的治疗，包括递减剂量的甲基强的松龙和4个疗程的利妥昔单抗(针对CD20蛋白的单抗)，AHA完全治愈，血压得到良好控制。结论。这份报告说明了一种罕见的AHA和BP的关联，支持了通过良好的短期治疗根除这种抑制剂的可能性。
Background. Acquired hemophilia A (AHA) is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Symptoms include severe and unexpected bleeding that may prove life-threatening. Case Study. We report a case of AHA associated with bullous pemphigoid (BP), a chronic, autoimmune, subepidermal, blistering skin disease. To our knowledge, this is the 25th documented case of such an association. Following treatment for less than 3 months consisting of methylprednisolone at decreasing dose levels along with four courses of rituximab (monoclonal antibody directed against the CD20 protein), AHA was completely cured and BP well-controlled. Conclusions. This report illustrates a rare association of AHA and BP, supporting the possibility of eradicating the inhibitor with a well-conducted short-term treatment.