Abstract Number: PB0492
Meeting: ISTH 2021 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Basic
Background: Haemophilia A is an X-linked inherited bleeding disorder caused by deficiency of coagulation factor VIII. Affected patients are predisposed to variety of bleeding events. There are limited number of publications regarding clinical characteristics and bleeding outcome of pediatric Hemophilia A in Oman.
Aims: This study aims to describe the clinical profile and outcome of Omani children with haemophilia A at the Royal Hospital, the largest referral centre in Oman, from 2006 to 2019.
Methods: This is a retrospective descriptive cohort study that includes all Omani children, younger than 13 years old, diagnosed with Hemophilia A and followed up at the Royal Hospital. Patients data was retrieved from their electronic chart system (AlShifa). Collected data includes age at presentation, factor VIII level, treatment, complications and bleeding episodes. Statistical analysis was performed using SPSS 24.0 (SPSS, Inc, Chicago, Illinois).
Results: Forty-four male patients were included with a mean age of 1 year (range 1 day – 9 years) at presentation. The mean period of follow up was 7.9 (SD 3.6) years. Twenty fours patients (55%) were diagnosed during screening test performed in response to a positive family history. The two most common complaints at presentation were muscle bleeding 14 (30%) and post circumcision-bleeding 5 (11%). According to factor’s level at diagnosis, 2 (5%) patients had mild disease, 29 (66%) patients had moderate disease, and 13 (30%) patients had severe disease. Twenty-eight patients (64%) were on regular prophylaxis. Ten (23%) patients developed target joint and six (14%) developed factor VIII inhibitors. The mean ABR outcome was 1.8, 4.6, and 4.6 for the mild, moderate, and severe patients respectively.
(Table 1)
| Mild | Moderate | Severe | |
| No. (%) | 2 (5%) | 29 (66%) | 13 (30%) |
| Mean duration of follow up | 8.0 years | 8.5 years | 6.7 years |
| Prophylaxis treatment No. (%) | 1 (50%) | 19 (66%) | 8 (62%) |
| Inhibitor development | 0 (0%) | 3 (10%) | 3 (23%) |
| Mean ABR | 1.8 | 4.6 | 4.6 |
| Patients with at least one target joint (%) | 0 (0%) | 7 (23%) | 3 (20%) |
Table 1: Summary of clinical phenotype and outcome for the cohort
Conclusions: The clinical phenotype of Omani children is apparently milder than what is published in the literature as there is higher percentage of moderate Hemophilia A but with a similar clinical outcome.
To cite this abstract in AMA style:
Al Rawahi A, Al Riyami F, Al Ghaithi I, Al Shukaili H, Al Abri M, Al Rawahi A. Clinical Profile and Bleeding Outcome of Pediatric Hemophilia A: The Royal Hospital’s Experience, Oman [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/clinical-profile-and-bleeding-outcome-of-pediatric-hemophilia-a-the-royal-hospitals-experience-oman/. Accessed November 29, 2021.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/clinical-profile-and-bleeding-outcome-of-pediatric-hemophilia-a-the-royal-hospitals-experience-oman/