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Screening for inhibitor development and it’s risk factors in patients with severe haemophilia a in Oman


Hematology & Transfusion International Journal
Hanan Fawzy Nazir, Department of Child Health, Sultan Qaboos University Hospital, Oman
Laila Al khanbashi, Department of Child Health, Oman Medical Speciality Board (OMSB), Oman
Yasser Ahmed Wali, Department of Pediatrics, Alexandria University, Egypt

Abstract

Introduction: A major challenging complication of factor VIII replacement therapy is the development of neutralizing antibodies, rendering therapy ineffective. There are recognized genetic and non-genetic risk factors for inhibitors development. This study aimed 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 included all patients with haemophilia A registered in Oman. Data were collected using computerized hospital information system. Patients’ 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 versus prophylaxis), type and dose of the concentrate, time interval of exposure to factor VIII till inhibitor development, previous surgeries or major bleeding events/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 21years (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 prophylaxis 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/Conclusion: Inhibitors development is a major complication in patients with hemophilia A in Oman. A larger scale study that includes all the registered patients and their genetic mutation is needed.

Keywords

severe hemophilia A, inhibitors, risk factors, QoL, hemarthrosis, PUP, HIS, SPSS

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