Abstract Number: PB1167
Meeting: ISTH 2020 Congress
Background: Rare bleeding disorders (RBDs) are defined as all hereditary coagulation disorders except Hemophilia A and B and Von Willebrand disease. In Iran, RBDs are more prevalent due to high rate of consanguineous marriage.
Aims: We aimed to update the frequency of different types of RBDs and their clinical characteristics and therapeutic aspects in Southern Iran until end of 2019.
Methods: In this cross-sectional study, 163 patients who have been registered as RBDs at Shiraz hemophilia comprehensive treatment center were evaluated. A designed questionnaire adapted from www.rbdd.org/pro-rbdd was used to gather required information.
Results: Mean age of patients was 22.9 ± 15.2 years (range: 1-75 years) and 55.2% of them were female. Most patients were in the age category of 19-40 years (Table 1). Distribution of different types of RBDs and their clinical characteristics are demonstrated in Table 2. The most frequent RBD was FVII deficiency (44 patients, 26.7%) and the most common presenting symptom was epistaxis (20.6%). Overall, most of the patients (40%) had mild factor deficiency (plasma factor concentrate >5%). Prophylaxis was given to 21.2% of all patients with satisfactory outcome.
Conclusions: RBDs are a heterogeneous group of disorders with a wide range of clinical manifestation. In contrary to hemophilia, the rate and severity of bleeding may not correlate well with plasma factor level in some types of RBDs (1). Since 2007, the frequency of patients with RBDs in Shiraz Hemophilia Center, has more than doubled (2). It may be explained by the high rate of consanguineous marriage in this region as well as higher detection rate. Prophylactic replacement therapy in a subgroup of patients especially FVII and FXIII deficiencies give rise to less bleeding episodes and higher quality of life (3).
To cite this abstract in AMA style:Bordbar M, Karimi M, Shahsavani A, Haghpanah S. Rare Bleeding Disorders in Southern Iran: Revisited and Updated Data [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/rare-bleeding-disorders-in-southern-iran-revisited-and-updated-data/. Accessed October 1, 2023.
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