Date Published: May 2, 2018
Author(s): Tarek Owaidah, Mahasen Saleh, Hazzah Alzahrani, Mahmood Abu-Riash, Ali Al Zahrani, Mohammed Almadani, Ayman Alsulaiman, Abdulmajeed Albanyan, Khawar Siddiqui, Khalid Al Saleh, Abdulkareem Al Momen.
Bleeding disorders vary in prevalence. While some are rare, some can be common in both sexes. Most bleeding disorders manifest as chronic bleeding tendencies or as an increase in bleeding during surgical procedures or trauma. The consequences of bleeding can be as simple as iron deficiency or catastrophic, resulting in severe morbidity and mortality. Bleeding disorders typically affect both sexes except hemophilia A and B, which mainly affects males.
We conducted a questionnaire-based survey among adolescents and young adults (1901 [49%] boys, 1980 [51%] girls) in Riyadh city regarding bleeding symptoms. Of these, 1849 (47.6%) responded “Yes/Positive” for at least one question about the bleeding symptoms.
The most common bleeding symptom was epistaxis (19.7% of the sample population) detected in Phase I of the study. A tandem survey was conducted among 525 adolescents who had responded “Yes/Positive” to any one of the questions inquiring about bleeding symptoms.
In this study, we report for the first time the prevalence of bleeding symptoms in a representative sample of Saudi adolescents and young adults.
Bleeding disorders are a group of inherited disorders with different prevalence rates depending on many ethnicities. The most known inherited bleeding disorders are hemophilia A and B, which are relatively rare. Hemophilia A affects 1 : 5000–10,000 males, while hemophilia B affects 1 : 50,000–100,000 males. Hemophilia A and B can be very serious and life-threatening for individuals as well as a costly disease for families and countries . von Willebrand disease (VWD) is another bleeding disorder, which is an inherited disorder that is caused by deficiency or dysfunction of VWF. VWD is a relatively common cause of bleeding, but the prevalence varies considerably among studies and depends strongly on the case definition that is used. The prevalence of VWD has been estimated in several countries on the basis of the number of symptomatic patients seen at hemostasis centers and ranges from about 23 to 110 per million population (0.0023–0.01%) . It is also been estimated by screening populations for bleeding symptoms (population-based approach), with estimates reported at 0.6%, 0.8%, and 1.3% [3–7]. These international estimates of the prevalence of VWD do not address ethnicity or geographic variables as potential independent factors, though ethnic variation in VWF levels can influence the diagnosis of VWD [8–10]. Moreover, most mild bleeding disorders are often unrecognized, as patients bleed only during stress periods or with surgery and medical procedures [11, 12]. The most common result of these chronic bleeds is iron deficiency anemia, which is more common in women due to excessive menstrual bleeding.
We conducted an epidemiological survey on a randomly selected Saudi national adolescent sample of intermediate and high school participants of both sexes in Riyadh using a semistructured validated condensed (MCMDM-1) VWD Bleeding Questionnaire. This questionnaire was selected owing to its capacity to generate quantifiable data from the entire study group . Process of translation into Arabic and adaptation of MCMDM-1 through an expert committee for implementation has been published elsewhere . A shorter questionnaire, derived from the same primary questions but with less detail, was extracted to be used as a primary screening tool for the initial phase of the study, whereas the original questionnaire was used in the second phase of the study only when participants gave a positive response to any primary question. The survey was conducted onsite by trained Arabic speaking interviewers. All questionnaires were coded for data entry. The process involved the following phases.
To our knowledge, this is the first largest screening study attempting to address the estimation of prevalence of symptoms of bleeding disorders in the capital city of Saudi Arabia. Prior studies from Saudi Arabia reporting the same have been smaller, hospital-based studies. For instance, El-Bostany et al.  assessed the local prevalence of some inherited bleeding disorders in pediatric patients which involved 43 children with various bleeding manifestations recruited from a children’s hospital in Cairo, Egypt, and Jeddah, Kingdom of Saudi Arabia. Of these, 12 (27.9%) had VWD, 11 (25.5%) had hemophilia A, three (7%) had hemophilia B, seven (16.3%) had platelet disorders, and 10 (23.3%) had bleeding of undiagnosed cause. In addition, Ahmed et al.  reported 34 cases of inherited bleeding disorders from Eastern Province of Saudi Arabia; of these, 15 had hemophilia, one had factor VII deficiency, one had factor X deficiency, 12 had Glanzmann thrombasthenia, and five had unidentified platelet function disorders. Moreover, Al-Sharif et al.  reported clinical phenotype of around 20 patients with factor XIII deficiency in the Riyadh region. Furthermore, Al-Fawaz et al.  conducted an 8-year retrospective analysis of patients referred for suspected inherited bleeding disorders in the Riyadh region and found 168 patients had bleeding symptoms that fulfilled the criteria for inherited bleeding disorders. Of these, 41 (24.4%) had hemophilia A, 16 (9.5%) had hemophilia B, 25 (14.9%) had VWD, 18 (10.7%) had Glanzmann thrombasthenia, 18 with Bernard-Soulier disease, five (3.0%) had factor XI deficiency, two (1.2%) had factor XII deficiency, four (2.4%) had factor V deficiency, four (2.4%) had factor VIII deficiency, one (0.6%) had factor VII deficiency, two (1.2%) had dysfibrinogenemia, and one (0.6%) had afibrinogenemia. Additionally, Islam and Quadri  conducted a 7-year retrospective review of all hospitals in Eastern Province of Saudi Arabia. They reported 54 patients diagnosed with hereditary coagulation factor deficiencies, including 42 hemophiliacs, 5 with probable factor XIII deficiency, and 7 with VWD. There are also rare reports from other Arab countries reporting small hospital-based studies [26–28].
This report is limited by the lack of laboratory related data further identifying specific bleeding disorders. It was beyond the feasibility of the research with respect to logistics and financial support. It would be very interesting to observe the prevalent forms of bleeding disorders in a future report hence guiding the policy makers for efficient resource allocation.
This survey which is the first epidemiological study for bleeding symptoms in Saudi Arabia using standardized tool (MCMDM-1) that had highlighted the need to conduct a national survey in the Kingdom on broader representative sample with extensive laboratory test to explore the prevalence of different bleeding disorders. We also recommend that physicians be cautious of the existence of bleeding disorders in the community as minor symptoms can get easily ignored and lead to a catastrophe when challenged by trauma or surgery. Also, a sustainable public awareness program focusing the early diagnosis, treatment, and genetic counseling among the residents of the regions with high prevalence of bleeding disorders should be initiated.