Abstract Number: PB0162
Meeting: ISTH 2020 Congress
Background: Acquired hemophilia (HA) is a rare bleeding disorder caused by autoantibodies against coagulation factors, especially factor VIII (FVIII). These inhibitors may induce severe bleeding episodes. It is necessary to establish hemostatic treatment and to eliminate the inhibitor using immunosuppressent drugs.
Aims: To describe the clinical characteristics and response to the treatment of the case series of HA of the Spanish registry to define new lines of knowledge on this topic.
Methods: In 2014 the Spanish registry of acquired hemophilia was activated. It is located on the SETH (Spanish Society of Thrombosis and Haemostasis) website. This registry has been implemented according to all national requirements and the Helsinki Declaration.
Results: 110 patients are registered (estimated incidence 200 cases), 53.6% of the subjects are male. Median age 74 years old (IQR, 63-84 years). Mean inhibitor titer is 17.6UB (IQR, 6.5- 44.6UB). 55.5% of patients have an underlying disease (40% autoimmune, 17% neoplasms, 13% postpartum). 98% of patients present bleeding at diagnosis: 43% mucocutaneous. 3 or 4 WHO grade bleedings are described in 16% of patients. Regarding to inhibitor eradication, 38% were treated with steroids and cyclophosphamide, 32% steroids, 22.7% rituximab associated with immunosuppressants and 7.3% steroids and cyclosporine.
Median follow-up of 9.5 months (IQR, 2 – 31 months), median time for inhibitor eradication is 1.5 months (IQR, 1-3 months). We do not find differences in time to eradication between treated or not with rituximab. 86% got response with the first line of treatment. Four patients relapsed, all of them within 2 months after treatment discontinuation. Mortality has been 17.5%: 47% due to infection/sepsis, 26% underlying disease, 11% hemorrhage and 11% angor.
Conclusions: Acquired hemophilia is underreported. The first cause of death is infectious, so initiatives to optimaze immunosuppressive treatment or infectious prophylaxis are necessary. We did not find differences in response rates between different eradication schemes.
To cite this abstract in AMA style:Mingot-Castellano ME, Bastida-Bermejo JM, Haya S, Pardos-Gea J, Fernandez-Sanchez de Mora MDC, Lluch R, Nuñez R, Alvarez-Roman MT, Soto-Ortega I, Martín-Antoran JM, Rodriguez-Garcia JA, Carrasco-Exposito M, Rodriguez-Alen JA, Cervero-Santiago C, Perez-Sanchez M, Rodriguez-Gonzalez R, Fernandez-Mosteirin N, Alonso-Escobar MN, Moreto-Quintana A, Marco-Vera P, Registro Español de Hemofilia Adquirida . Acquired Haemophilia Spanish Registry, First Five Years of Activity [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/acquired-haemophilia-spanish-registry-first-five-years-of-activity/. Accessed March 3, 2021.
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