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- Title
Screening for inhibitors development and it's risk factors in patients with severe haemophilia in Oman.
- Authors
AL Khanbashi, Laila; Al Jamei, Adawiya; Nazir, Hanan; Wali, Yasser; Al Kamzari, Maryam; Al Belushi, Noof
- Abstract
Introduction: A major challenging complication of factor VIII replacement therapy is the development of neutralizing antibodies, rendering therapy ineffective. There are recognised genetic and non-genetic risk factors for inhibitors development. This study aims to screen for the prevalence of inhibitor development among Omani patients with severe haemophilia A and to define its non-genetic risk factors that might help to develop prevention protocols. Methods: A Retrospective cohort study that includes all patients with haemophilia A registered in Oman. Data were collected using hospital information system computerized data. Patient's demographic data: MRN, age at diagnosis, age at first treatment, date of the first documented inhibitor development, & data on non -genetic risk factors such as, mode of treatment (on demand Vs prophylaxis) type and dose of the concentrate, time interval of exposure to factor VIII till inhibitor development, previous surgeries or major bleeding events or blood transfusion; were included. Missing data were collected through phone interviews whenever possible. Results: Out of the 156 patients registered in Oman, 78 patients had complete data. The age of inhibitor development ranged between 16 months and 21 years (109.4 + 79.9 months). The prevalence of inhibitors among our patients was 35% and mainly of low titer 87%. There was a significant correlation between the intensity of first treatment and inhibitor development (p < 0.05). Inhibitor development was not associated with type of the factor used, time interval of exposure to factor VIII, use of on-demand or prophylaxis regimen and history of major bleed or previous surgery. Discussion and Conclusion: Inhibitors development is a major complication in patients with hemophilia A in Oman. The reported prevalence matches the international reported figures. A larger scale study that includes all the registered patients and their genetic mutation is needed.
- Publication
Journal of Applied Hematology, 2019, Vol 10, pS42
- ISSN
1658-5127
- Publication type
Article