期刊论文详细信息
Journal of Clinical Medicine
Acquired Haemophilia A: A 15-Year Single-Centre Experience of Demography, Clinical Features and Outcome
Nora Butta Coll1  Víctor Jiménez-Yuste1  Raisa Guerrero Camacho1  Mónica Martín Salces1  Damaris Zagrean1  María Teresa Álvarez Román1  Isabel Rivas Pollmar1  Mercedes Gasior Kabat1 
[1] Servicio de Hematología y Hemoterapia, Hospital Universitario La Paz, Paseo de la Castellana 231, 28046 Madrid, Spain;
关键词: acquired;    haemophilia A;    demography;    presentation;   
DOI  :  10.3390/jcm11102721
来源: DOAJ
【 摘 要 】

Acquired haemophilia A (AHA) is a rare severe bleeding disorder resulting from the production of autoantibodies directed against coagulation factor VIII. At presentation, bleeding events can be severe, and an early diagnosis and treatment are of major importance. The current study aims to analyse the treated patients who have been diagnosed with AHA for a better understanding of our population and treatment outcome. We conducted a retrospective study with 26 patients who had been diagnosed with AHA and who were treated in our hospital between January 2006 and January 2021. The patients ranged in age from 30 to 85 years old: 46.10% were men, 46.10% had no known underlying condition, 27% had an underlying malignancy, 7.60% presented with other diseases: psoriatic arthritis and Paget’s disease, and 19.30% presented with AHA during puerperium. All of the patients had bleeding events and were treated with bypass agents for this as well as with immunosuppressive therapy to eradicate the inhibitor. A total of 53.80% of the patients had major bleeding. Sixty-nine percent of the patients achieved complete remission, but 26.90% died during the follow-up, although bleeding was not the cause of death in any of these cases. Our observations underline the importance of clinical suspicion and early referral to centres with experience and laboratory facilities for managing AHA.

【 授权许可】

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