Abstract Number: PB0778
Meeting: ISTH 2020 Congress
Background: Acquired hemophilia (AH) is a rare coagulation disorder whose impact is sometimes underestimated by the lack of adequate records and its diagnostic complexity. Its importance lies in the high mortality rate.
Aims: Analyze the clinical profile and analytical parameters of patients diagnosed with AH in a third level hospital.
Methods: Retrospective observational study of patients diagnosed with AH in the Hematology Department of Miguel Servet University Hospital, from 2008 to 2019. Demographic variables, clinical characteristics, laboratory parameters and treatment received have been analyzed.
Results: 8 patients were included, 6 men and 2 women with a middle age of 74,89 years (extremes 47-97; DS 14,45). All patients presented more than one bleeding episode, in total 17, classified according to location (table). About the etiology, 3 patients presented secondary AH (lynfoproliferative syndrome, bullous pemphigus o psoriasis) and 5 had idiopathic HA. With regard to erradication treatment: 4 patients received corticosteroids, 3 patients received corticosteroids and cyclophosphamine and 1 patient received rituximab. Six patients needed by-pass agents, 5 of them with rFVIIa and 1 needed sequential treatment with rFVIIa and aCCP. Besides, 6 patients required transfusion of red blood cell concentrates. Of the 8 registered patients, one patient was hospitalized because of an urinary infection. Two patients died, one in the context of intracranial bleeding and another due to complications of cardiomyopathy. Analytically the hemostasis parameters were: median aPTT of 65,2 seconds (extremes: 53,3-160,9) and a median level of FVIII at diagnose of 3% (extremes: 0,1-25). Finally, the median inhibitory tittle at diagnose was of 8 Bethesda units (BU) (extremes: 8-65).
Conclusions: Acquired hemophilia is a life-threatening clinical situation. Immunosuppressive treatment achieves overall response rates in 70-80% of patients. In our series, the clinical hemorrhagic profile, the observed mortality, as well as the rate of responses to immunosuppressive treatment were similar to those described in the literature.
|Number of bleeding episodes||8||5||1||1||1||1|
[Number and location of bleeding episodes]
To cite this abstract in AMA style:Gómez Martínez A, Villalba Montaner M, Salvador Rupérez E, García Ortego A, Pinzón Mariño S, Martín-Consuegra Ramos S, Hernández Mata CF, Moreno Carbonell M, González Gómez E, Calvo Villas JM, Fernández Mosteirín N, Delgado Beltrán P. Analysis of Patients with Diagnosis of Adquired Haemophilia in a Third Level Hospital [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/analysis-of-patients-with-diagnosis-of-adquired-haemophilia-in-a-third-level-hospital/. Accessed January 28, 2022.
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