BACKGROUND Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disordercaused by circulating autoantibodies against factor Ⅷ (FⅧ). In approximately50% of the patients, the condition is associated w...BACKGROUND Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disordercaused by circulating autoantibodies against factor Ⅷ (FⅧ). In approximately50% of the patients, the condition is associated with autoimmune diseases,cancers, medication use, pregnancy, and the post-partum period. Bullous pemphigoid(BP) is a chronic autoimmune subepidermal blistering disease associatedwith tissue-bound and circulating autoantibodies against BP antigens 180 (BP180)and 230 (BP230). AHA-associated BP has a high mortality rate;hence, the understandingof this disease must improve.CASE SUMMARY A 69-year-old man presented with erythema, blisters, blood blisters, and crustsaccompanied by severe pruritus for more than 20 days, and ecchymosis andswelling on his left upper arm for 3 days. Pathological examination revealed asubepidermal blister that contained eosinophils. Laboratory tests showed that theBP180 autoantibody levels had increased, isolated activated partial thromboplastintime was notably prolonged (115.6 s), and coagulation FⅧ activity wasextremely low (< 1.0%). Furthermore, the FⅧ inhibitor titer had greatlyincreased (59.2 Bethesda units). Therefore, the patient was diagnosed as having BP associated with AHA, prescribed 0.05% topical halometasone cream, and transferred to a higher-level hospitalfor effective treatment;however, he died after 2 days.CONCLUSION AHA associated BP is rare, dangerous, and has a high mortality rate. Therefore, its timely diagnosis and effectivetreatment are necessary.展开更多
目的了解中国大陆血友病患者(people with hemophilia,PWH)的治疗现状及经济负担,并探讨血友病在中国的最佳治疗方案。方法于2011年6月系统检索《中国生物医学文献数据库》(CBM)、《中国期刊全文数据库》(CNKI)、《中国科技期刊数据库》...目的了解中国大陆血友病患者(people with hemophilia,PWH)的治疗现状及经济负担,并探讨血友病在中国的最佳治疗方案。方法于2011年6月系统检索《中国生物医学文献数据库》(CBM)、《中国期刊全文数据库》(CNKI)、《中国科技期刊数据库》(VIP)、万方数据库等中文数据库,以及PubMed、EMbase和The Cochrane Library(2011年第6期)等英文数据库中收录的1980～2011年发表的有关PWH治疗及经济负担的研究。结果中国PWH诊断、治疗滞后;超30%的PWH不治疗或偶尔治疗,不足10%的PWH接受预防治疗;仍有相当一部分患者使用新鲜冷冻血浆(FFP)或冷沉淀等容易导致血源性病毒传播疾病的传统血制品;大于50%的PWH家庭只能支付很少或完全支付不起医疗费用。低剂量预防治疗相对于足量按需治疗更经济。以目前中国PWH实际治疗情况计算,每例PWH每年多投入53 844元即可实现低剂量预防治疗,从而减少80%的出血。结论中国大陆PWH的治疗现状差,经济负担重,推行综合关怀模式,采用低剂量预防治疗是最佳治疗方案。展开更多
基金Supported by Traditional Chinese Medicine Research Program of Hebei Provincial Administration of Traditional Chinese Medicine,No.2025313 and No.2025448.
文摘BACKGROUND Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disordercaused by circulating autoantibodies against factor Ⅷ (FⅧ). In approximately50% of the patients, the condition is associated with autoimmune diseases,cancers, medication use, pregnancy, and the post-partum period. Bullous pemphigoid(BP) is a chronic autoimmune subepidermal blistering disease associatedwith tissue-bound and circulating autoantibodies against BP antigens 180 (BP180)and 230 (BP230). AHA-associated BP has a high mortality rate;hence, the understandingof this disease must improve.CASE SUMMARY A 69-year-old man presented with erythema, blisters, blood blisters, and crustsaccompanied by severe pruritus for more than 20 days, and ecchymosis andswelling on his left upper arm for 3 days. Pathological examination revealed asubepidermal blister that contained eosinophils. Laboratory tests showed that theBP180 autoantibody levels had increased, isolated activated partial thromboplastintime was notably prolonged (115.6 s), and coagulation FⅧ activity wasextremely low (< 1.0%). Furthermore, the FⅧ inhibitor titer had greatlyincreased (59.2 Bethesda units). Therefore, the patient was diagnosed as having BP associated with AHA, prescribed 0.05% topical halometasone cream, and transferred to a higher-level hospitalfor effective treatment;however, he died after 2 days.CONCLUSION AHA associated BP is rare, dangerous, and has a high mortality rate. Therefore, its timely diagnosis and effectivetreatment are necessary.
