Acquired Haemophilia A: A 15-Year Single-Centre Experience of Demography, Clinical Features and Outcome

被引:7
作者
Guerrero Camacho, Raisa [1 ,4 ]
Alvarez Roman, Maria Teresa [1 ,2 ,3 ]
Butta Coll, Nora [1 ,2 ]
Zagrean, Damaris [1 ]
Rivas Pollmar, Isabel [1 ,2 ]
Martin Salces, Monica [1 ,2 ]
Gasior Kabat, Mercedes [1 ]
Jimenez-Yuste, Victor [1 ,2 ,3 ]
机构
[1] Hosp Univ La Paz, Serv Hematol & Hemoterapia, Paseo La Castellana 231, Madrid 28046, Spain
[2] IdiPAZ, Coagulopathies & Haemostasis Disorders Grp, Paseo Castellana 231, Madrid 28046, Spain
[3] Univ Autonoma Madrid, Fac Med, Arzobispo Morcillo 4, E-28029 Madrid, Spain
[4] Blackpool Teaching Hosp NHS Fdn Trust, Blackpool FY3 8NR, England
关键词
acquired; haemophilia A; demography; presentation; FACTOR-VIII; MANAGEMENT; INHIBITORS; RITUXIMAB; SURVEILLANCE; DIAGNOSIS; COHORT;
D O I
10.3390/jcm11102721
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
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.
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