Winfield Laramie S, Brooks Marjory B
Steinbeck Country Equine Clinic, 15881 Toro Hills Ave, Salinas, CA 93908.
J Am Vet Med Assoc. 2014 Mar 15;244(6):719-23. doi: 10.2460/javma.244.6.719.
A 23-year-old Thoroughbred mare was evaluated because of a coagulopathy causing hemoperitoneum, hematomas, and signs of blood loss-induced anemia.
The mare had tachycardia, pallor, hypoperfusion, and a large mass in the right flank. The mass was further characterized ultrasonographically as an extensive hematoma in the body wall with associated hemoabdomen. Coagulation testing revealed persistent, specific prolongation of the activated partial thromboplastin time (> 100 seconds; reference interval, 24 to 44 seconds) attributable to severe factor VIII deficiency (12%; reference interval, 50% to 200%). On the basis of the horse's age, lack of previous signs of a bleeding diathesis, and subsequent quantification of plasma factor VIII inhibitory activity (Bethesda assay titer, 2.7 Bethesda units/mL), acquired hemophilia A was diagnosed. The medical history did not reveal risk factors or underlying diseases; thus, the development of inhibitory antibodies against factor VIII was considered to be idiopathic.
The mare was treated with 2 transfusions of fresh whole blood and fresh-frozen plasma. Immunosuppressive treatment consisting of dexamethasone and azathioprine was initiated. Factor VIII deficiency and signs of coagulopathy resolved, and the inhibitory antibody titer decreased. The mare remained healthy with no relapse for at least 1 year after treatment.
Horses may develop inhibitory antibodies against factor VIII that cause acquired hemophilia A. A treatment strategy combining transfusions of whole blood and fresh-frozen plasma and administration of immunosuppressive agents was effective and induced sustained remission for at least 1 year in the mare described here.
一匹23岁的纯种母马因凝血功能障碍导致腹腔积血、血肿和失血所致贫血体征而接受评估。
该母马出现心动过速、面色苍白、灌注不足,右侧胁腹有一巨大肿块。超声检查进一步确定该肿块为体壁广泛血肿并伴有腹腔积血。凝血检测显示活化部分凝血活酶时间持续、特异性延长(>100秒;参考区间为24至44秒),原因是严重的凝血因子VIII缺乏(12%;参考区间为50%至200%)。根据该马的年龄、既往无出血素质体征以及随后血浆凝血因子VIII抑制活性的定量分析(贝塞斯达试验滴度为2.7贝塞斯达单位/毫升),诊断为获得性血友病A。病史未显示危险因素或潜在疾病;因此,针对凝血因子VIII的抑制性抗体的产生被认为是特发性的。
给该母马输注了2次新鲜全血和新鲜冰冻血浆。开始使用地塞米松和硫唑嘌呤进行免疫抑制治疗。凝血因子VIII缺乏和凝血功能障碍体征得到缓解,抑制性抗体滴度下降。治疗后该母马至少1年保持健康且无复发。
马可能产生针对凝血因子VIII的抑制性抗体,导致获得性血友病A。在此描述的母马中,一种结合输注全血和新鲜冰冻血浆以及给予免疫抑制剂的治疗策略是有效的,并诱导了至少1年的持续缓解。