Mameli Antonella, Marongiu Francesco, Fenu Lara, Ruberto Maria Filomena, Schirru Paola, Cornacchini Simona, Barcellona Doris
Azienda Ospedaliera Universitaria di Cagliari, Haemostasis and Thrombosis Unit, Cagliari, Italy
University of Cagliari Faculty of Medicine, Department of Medical Sciences and Public Health, Cagliari, Italy
Turk J Haematol. 2024 Dec 2;41(4):264-270. doi: 10.4274/tjh.galenos.2024.2024.0230. Epub 2024 Oct 28.
Acquired hemophilia A (AHA) is a rare autoimmune disease characterized by the presence of autoantibodies against coagulation factor VIII, leading to spontaneous hemorrhage in patients without a prior family or personal history of bleeding. This study describes the demographics, diagnosis, underlying disorders, bleeding characteristics, treatment, and outcomes of 41 AHA patients together with specific case reports.
Diagnosis and treatment of these patients occurred between 2005 and 2023. The median age at diagnosis was 67.8 (range: 15-93) years. Among the 41 patients, 10 (24%) cases were idiopathic, 4 (10%) were postpartum, 18 (44%) involved autoimmune diseases, and 9 (22%) involved a diagnosis of cancer.
The diagnostic delay exceeded 30 days in 15 of the 41 cases (36.5%). A total of 38 of the 41 (93%) patients presented with spontaneous bleeding, with mucocutaneous bleeding being the most common presentation (23/41, 56%). Four patients experienced postpartum bleeding. Clinical remission was achieved by 100% of patients and no patients died.
Hemostatic and immunosuppressive therapy is essential in AHA, and it should be started as soon as possible in patients with bleeding. However, a significant delay in diagnosis was observed in these cases. The absence of mortality is likely attributable to the management of the disease within a specialized hemostasis and thrombosis unit, which offers a clinical ward, a specialized laboratory, and a dedicated ambulatory service. The Italian Society for the Study of Haemostasis and Thrombosis is working to secure recognition of this essential role in every hospital.
获得性血友病A（AHA）是一种罕见的自身免疫性疾病，其特征是存在针对凝血因子VIII的自身抗体，导致无家族或个人出血史的患者发生自发性出血。本研究描述了41例AHA患者的人口统计学特征、诊断、基础疾病、出血特征、治疗及预后，并伴有具体病例报告。
这些患者的诊断和治疗时间为2005年至2023年。诊断时的中位年龄为67.8岁（范围：15 - 93岁）。41例患者中，10例（24%）为特发性，4例（10%）为产后发病，18例（44%）伴有自身免疫性疾病，9例（22%）伴有癌症诊断。
41例中有15例（36.5%）诊断延迟超过30天。41例患者中有38例（93%）出现自发性出血，黏膜皮肤出血是最常见的表现（23/41，56%）。4例患者发生产后出血。所有患者均实现临床缓解，无患者死亡。
止血和免疫抑制治疗在AHA中至关重要，对于出血患者应尽早开始治疗。然而，这些病例中观察到明显的诊断延迟。无死亡病例可能归因于在专门的止血和血栓形成单元内对该疾病的管理，该单元提供临床病房、专门实验室和专门的门诊服务。意大利止血与血栓形成研究协会正在努力确保在每家医院都能认识到这一重要作用。
