Chen Victor, Roby Lauren C, Wentzel Stephanie, Li Mingjia, Jones Nicholas
The Ohio State University College of Medicine, Columbus, OH, USA.
The Ohio State University Hematology-Oncology Fellowship, Columbus, OH, USA.
J Med Case Rep. 2022 May 3;16(1):177. doi: 10.1186/s13256-022-03402-x.
Acquired hemophilia A is a disorder caused by autoantibodies against coagulation factor VIII that may present with severe bleeding. We report a rare case of acquired hemophilia A presenting with coexisting lupus anticoagulant.
An 81-year-old Caucasian female presented with large ecchymoses over the torso and extremities in the setting of an enoxaparin bridge to warfarin. Anticoagulation was held, but she continued to develop bruises with significant anemia and prolonged coagulation studies that failed to correct with mixing. Workup revealed factor VIII activity < 1% and a positive lupus anticoagulant. Initial testing for a factor VIII inhibitor was confounded by the presence of lupus anticoagulant, requiring a chromogenic Bethesda assay to confirm the presence of the inhibitor, establishing the diagnosis of acquired hemophilia A. The patient was initially treated with oral prednisone 80 mg daily and factor VIII inhibitor bypassing activity 25 units/kg twice daily before transitioning to susoctocog alfa 50 units/kg twice daily after placement of a tunneled line for outpatient rituximab infusions. On discharge, the patient's ecchymoses were resolving and factor VIII levels improved. Following completion of rituximab therapy, the patient's factor VIII activity normalized and factor VIII inhibitor was suppressed.
Diagnosis of acquired hemophilia A can be confounded by other causes of abnormal coagulation studies and may require specialized testing, such as a chromogenic Bethesda assay, to confirm the presence of a factor VIII inhibitor.
获得性血友病A是一种由针对凝血因子VIII的自身抗体引起的疾病，可能表现为严重出血。我们报告一例罕见的获得性血友病A合并狼疮抗凝物的病例。
一名81岁的白种女性在从依诺肝素过渡到华法林的过程中，躯干和四肢出现大片瘀斑。抗凝治疗暂停，但她仍持续出现瘀斑，并伴有严重贫血和凝血检查延长，混合试验无法纠正。检查发现因子VIII活性<1%，狼疮抗凝物阳性。狼疮抗凝物的存在使最初的因子VIII抑制剂检测结果混淆，需要进行发色底物贝塞斯达试验来确认抑制剂的存在，从而确诊获得性血友病A。患者最初接受每日80毫克口服泼尼松和每日两次25单位/千克的因子VIII抑制剂旁路活性治疗，在置入隧道式导管用于门诊利妥昔单抗输注后，改为每日两次50单位/千克的苏托考格α治疗。出院时，患者的瘀斑正在消退，因子VIII水平有所改善。利妥昔单抗治疗完成后，患者的因子VIII活性恢复正常，因子VIII抑制剂受到抑制。
获得性血友病A的诊断可能因其他凝血检查异常原因而混淆，可能需要进行专门检测，如发色底物贝塞斯达试验，以确认因子VIII抑制剂的存在。
