RARE BLEEDING DISORDERS: SPECTRUM OF DISEASE AND CLINICAL MANIFESTATIONS IN KING FAHAD ARMED FORCES HOSPITAL OF JEDDAH, SAUDI ARABIA

*Alia A. Abotaleb MD, Consultant Hematology‎, Abdulrahim M. Basendwah MD, Hematology Consultant, Iman A. Alhazmi MD, Consultant Hematology, Samy M. Attalah MD, Consultant Hematopathologist, Ehab M. Taha, Shabana A. Kamal, Maha M. Alshareef, Duaa A. Khan‎, Sumaia A. Felimban‎, Naif Aljohani, Bushra Al Qadi, Samah A. Mokhlis MsC

ABSTRACT

Background: Rare bleeding disorders (RBDs) are heterogeneous disorders, mostly inherited in an autosomal recessive pattern. The clinical picture of RBDs is highly variable making diagnosis and best treatment quite challenging. Current treatment is based on both replacement therapy and non-trans fusional treatment. Objectives: In this study, we present prevalence, clinical presentation, and management of Saudi patients with RBDs at King Fahad Armed Forces Hospital (KFAFH), Jeddah, Saudi Arabia. The study findings will be useful to set up a database of these rare bleeding disorders in our institute, which will help clinicians in the early diagnosis and management. Methods: In this retrospective case review, detailed clinical information’s was extracted from medical records in all patients who had rare clotting factor deficiency at (KFAFH) over the period of (2013-2020). Results: Among 1,581 patients with suspected coagulation disorders, 639 (40.41%) had a bleeding disorder. 103(16.11%) had a rare bleeding disorder; with a male-to-female ratio of 1.01. The median patient age was 5 years. The most common disorder was factor VII deficiency (50 patients, 48.5%). The most common clinical presentation was epistaxis in 15 (14.56%) and bruising in 13 (12.62%) patients.

Keywords: Rare Bleeding Disorders, Factor Deficiency, Factor assay, Saudi Arabia, Inherited Bleeding Disorders.