Abstract Number: PB0899
Meeting: ISTH 2021 Congress
Background: Inherited platelet disorders (IPDs) are a heterogeneous group of disorders associated with bleeding diatheses of varying severities. Light Transmission Aggregometry (LTA) is the most widely used method for the diagnosis of IPDs which monitor the responses of platelet to a panel of agonists. IPDs are considered to be rare with a frequency of 1: 10 000 of healthy population. To date, very little is known about the prevalence of IPD within the Omani population.
Aims: We therefore aimed to investigate the epidemiology of IPD among 246 patients (75 male and 171 female, median age 20±16) referred to the Royal Hospital between 2006 and 2020.
Methods: Data files of 246 patients were analyzed for the following parameters: LTA (ADP, Epinephrie, Arachidonic acid, Collagen and ristocetin) PFA-100 closure times (CT) and flow cytometry. Patients were categorized on the basis of the observed defects.
Results: Overall, IPD was diagnosed in 50(20.3%) patients, in which, 15 patients were identified with secretion defects, 12 patients with Gi defects, 8 patients with Glanzmann’s thrombasthenia (GT), 4 patients with cyclooxygenase (Cox) defects, 7 patients with multiple defects and 4 patients with other defects. Platelet count and Platelet Mean Volume (mean ± SD) in patients’ whole blood were 273±146 x 103/µL and 8.7±2 fl, respectively. PFA-100 was tested in 36/50 patients identified to have IPD of which 69% (25) gave abnormal CT. Flow cytometry results tested on patients with GT showed lack of expression of CD41 and CD61 on platelet surface.
Conclusions: Our current study revealed that secretion defect is the most common type of IPDs among Omani patients. The number of patients identified with GT in this study is in line with previously reported studies within the GCC countries.
To cite this abstract in AMA style:Al Ghaithi R, Al Hashami S, Al Amri N, Al Shiyadi S, Al Lawati R, Al Busaidi R, Ambusaidi H, Al Lamki S, Al Yahyai M, Al Riyami M, Al Musalhi M, Al Salmi I. Investigation of the Epidemiology of Inherited Platelet Disorders in Patients Attending the Royal Hospital, Muscat, Oman [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 1). https://abstracts.isth.org/abstract/investigation-of-the-epidemiology-of-inherited-platelet-disorders-in-patients-attending-the-royal-hospital-muscat-oman/. Accessed September 16, 2021.
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