Mingot-Castellano María-Eva, Pardos-Gea Josep, Haya Saturnino, Bastida-Bermejo José-María, Tàssies Dolors, Marco-Rico Ana, Núñez Ramiro, García-Candel Faustino, de Mora María-Carmen Fernández-Sanchez, Soto Inmaculada, Álvarez-Román María-Teresa, Asenjo Susana, Carrasco Marina, Lluch-García Rafael, Martín-Antorán José-Manuel, Rodríguez-Alén Agustín, Roselló Elena, Torres-Miñana Laura, Marcellini-Antonio Shally, Moretó-Quinana Ana, Rodríguez-García José-Antonio, Aguinaco-Culebras Reyes, Alonso-Escobar Nieves, Cervero-Santiago Carlos, Fernández-Mosteirín Núria, Martínez-Badás María-Paz, Pérez-Sánchez Montserrat, Pérez-Montes Rocío, Rodríguez-González Ramón, Uribe-Barrientos Marisol, Caparrós-Miranda Isabel Socorro, Iglesias-Fernández Miriam, Baena Ángela, Rodríguez-López Manuel, Sebrango-Sandia Ana, Vázquez-Fernández Irene, Marco Pascual
Hospital Universitario Virgen del Rocío, Sevilla, Spain.
Hospital Regional Universitario de Málaga, Málaga, Spain.
Blood Adv. 2021 Oct 12;5(19):3821-3829. doi: 10.1182/bloodadvances.2021004626.
The Spanish Acquired Hemophilia A (AHA) Registry is intended to update the status of AHA in Spain. One hundred and fifty-four patients were included and retrospectively followed for a median of 12 months. Patients were predominantly male (56.3%), with median age at diagnosis of 74 years. AHA was more frequently idiopathic (44.1%) and autoimmune disorder-associated (31.7%). Thirty-four percent of patients were on antithrombotic therapy at diagnosis. Hemostatic treatment was used in 70% of patients. Recombinant activated factor VII was more frequently infused (60.3% vs 20.6% activated prothrombin complex concentrate). Only 1 patient did not achieve control of hemorrhage. Complete remission (CR) was achieved by 84.2% of cases after immunosuppressive therapy. Steroids alone were less efficient than the other strategies (68.2% vs 87.2%, P = .049), whereas no differences existed among these (steroids/cyclophosphamide, 88.5%, vs steroids/calcineurin inhibitors, 81.2%, vs rituximab-based regimens, 87.5%). Female sex and high inhibitor levels influenced CR negatively. Thirty-six deaths (23.8%) were reported. Main causes of death were infection (15 patients, 9.9%) and hemorrhage (5 patients, 3.3%). All hemorrhage-related and half the infection-related deaths occurred within 2 months of diagnosis. Prior antithrombotic therapy was inversely associated with survival, irrespective of age. Median age of nonsurvivors was significantly higher (79 vs 73 years in survivors). Patients dying of infection were older than the other nonsurvivors (85 vs 78 years). In summary, fatal infection in the first months is common in our series. Antithrombotic therapy is associated with mortality. Particular care should be taken to avoid misdiagnosis.
西班牙获得性甲型血友病（AHA）登记旨在更新西班牙AHA的状况。纳入了154例患者，并进行了中位时间为12个月的回顾性随访。患者以男性为主（56.3%），诊断时的中位年龄为74岁。AHA更常见于特发性（44.1%）和自身免疫性疾病相关（31.7%）。34%的患者在诊断时接受抗血栓治疗。70%的患者使用了止血治疗。重组活化因子VII的输注更为频繁（60.3%对活化凝血酶原复合物浓缩物的20.6%）。只有1例患者未实现出血控制。免疫抑制治疗后84.2%的病例实现了完全缓解（CR）。单独使用类固醇的疗效低于其他策略（68.2%对87.2%，P = 0.049），而这些策略之间无差异（类固醇/环磷酰胺，88.5%，对类固醇/钙调神经磷酸酶抑制剂，81.2%，对基于利妥昔单抗的方案，87.5%）。女性和高抑制剂水平对CR有负面影响。报告了36例死亡（23.8%）。主要死亡原因是感染（15例患者，9.9%）和出血（5例患者，3.3%）。所有与出血相关的死亡以及一半与感染相关的死亡发生在诊断后2个月内。无论年龄如何，先前的抗血栓治疗与生存率呈负相关。非幸存者的中位年龄显著更高（79岁对幸存者的73岁）。死于感染的患者比其他非幸存者年龄更大（85岁对78岁）。总之，在我们的系列中，最初几个月的致命感染很常见。抗血栓治疗与死亡率相关。应特别注意避免误诊。
