Zaidi Abdul Rehman Z, AlSheef Mohammed, Motabi Ibraheem H, Zaidi Syed Ziauddin A, Tailor Imran K
Internal Medicine, King Fahad Medical City, Riyadh, SAU.
Medicine, King Fahad Medical City, Riyadh, SAU.
Cureus. 2019 May 30;11(5):e4786. doi: 10.7759/cureus.4786.
Acquired hemophilia is a rare autoimmune disorder that is a result of antibodies against clotting factor VIII and it presents with excessive or prolonged bleeding, often into the muscles. Thrombotic phenomena with lupus anticoagulant are common in patients with systemic lupus erythematosus (SLE). We report a rare case of a young female with no significant past medical history presenting with hematoma of the hand who was later on found to have acquired hemophilia, SLE with antiphospholipid antibodies (APLA). She was successfully treated with upfront rituximab and prednisolone leading to early and prolonged remission. No increased incidence of infections was noted. Upfront rituximab appears to be a safe and effective option in the management of such patients when compared to use of cytotoxic agents such as cyclophosphamide; however, further data from randomized studies is needed. Neutropenia and acquired hemophilia should also be considered to be listed under hematological manifestations of SLE diagnostic criteria, as they are not uncommon in such patients.
获得性血友病是一种罕见的自身免疫性疾病，是由抗凝血因子VIII抗体引起的，表现为过度出血或出血时间延长，常发生于肌肉内。狼疮抗凝物导致的血栓形成现象在系统性红斑狼疮（SLE）患者中很常见。我们报告了一例罕见病例，一名既往无重大病史的年轻女性出现手部血肿，后来被发现患有获得性血友病、伴有抗磷脂抗体（APLA）的SLE。她接受了利妥昔单抗和泼尼松龙的初始治疗并获得成功，实现了早期且持久的缓解。未观察到感染发生率增加。与使用环磷酰胺等细胞毒性药物相比，初始使用利妥昔单抗似乎是治疗此类患者的一种安全有效的选择；然而，需要来自随机研究的进一步数据。中性粒细胞减少症和获得性血友病也应被视为列入SLE诊断标准的血液学表现，因为它们在这类患者中并不罕见。
