CC BY 4.0 · TH Open 2020; 04(04): e457-e462
DOI: 10.1055/s-0040-1721500
Original Article

Prevalence of Coagulation Factors Deficiency among Young Adults in Saudi Arabia: A National Survey

Khalid A. AlSaleh
1   Department of Medicine, King Saud University Medical City, King Saud University, Riyadh, Saudi Arabia
,
2   Department of Genetics, King Faisal Specialist Hospital and Research Centre, and College of Medicine, Alfaisal University, Riyadh, Saudi Arabia
,
Ayman AlSuliman
3   Research Center, King Faisal Specialist Hospital, Alfaisal University, Riyadh, Saudi Arabia
,
Mohammed Zolaly
4   Department of Pediatric, Taibah University, Medina, Saudi Arabia
,
Abdul Majeed Albanyan
5   College of Medical Applied Sciences, King Saud University, Riyadh, Saudi Arabia
,
Nouf AlOtaishan
2   Department of Genetics, King Faisal Specialist Hospital and Research Centre, and College of Medicine, Alfaisal University, Riyadh, Saudi Arabia
,
Esra Abudouleh
5   College of Medical Applied Sciences, King Saud University, Riyadh, Saudi Arabia
,
Nervana Bayoumy
6   Department of Physiology, College of Medicine, King Saud University, Riyadh, Saudi Arabia
,
Ahmad Tarawah
7   Medina Maternity and Children Hospital, Medina, Saudi Arabia
,
Faisal AlZahrani
8   Department of Pathology, Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia
,
Faisal AlAllaf
9   Department of Molecular Medicine, Umm Al Qura University, Makkah, Saudi Arabia
,
Abdul kareem AlMomen
10   Department of Pathology, College of Medicine, King Saud University, Riyadh, Saudi Arabia
,
Raihan Sajid
11   Department of Pathology, Alfaisal University, Riyadh, Saudi Arabia
,
12   Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Alfaisal University, Riyadh, Saudi Arabia
› Author Affiliations
Funding This study was supported by KACST (408–34) RAC KFSHRC (2130036).

Abstract

Introduction Inherited bleeding disorders vary in prevalence due to genetic disparity and ethnicity. Little is known about the prevalence of coagulation factor deficiency and bleeding disorders in middle-eastern population.

Methods Young Saudi adults with at least one positive bleeding symptom reported in semi-structured validated condensed MCMDM-1vWD questionnaire were tested for complete blood count, routine and special coagulation tests, serum ferritin level, and capillary zone electrophoresis. After initial testing, those with prolonged prothrombin time (PT) or activated prothrombin time (APTT) had further testing to evaluate coagulation factors level. Platelet function was tested through platelet function analyzer (PFA)-100, and multiplate aggregometer (MEA) on patients suspected of having platelet disorders.

Results Six-hundred-forty patients (male = 347, 54.2%) were included. A possible platelet function defect was diagnosed in three patients with one matching Glanzmann's thrombasthenia trait pattern, and one that of Bernard-Soulier trait pattern. One patient was diagnosed with von Willebrand disease. Deficiencies in coagulation factor levels were revealed as F-VIII in 14 (7.4%), F-IX in 15 (7.6%), F-II in two (3.3%), F-V in 17 (26.1%), FVII in two (3.1%), and F-X in one (1.8%) of study subjects; low vWF activity (<50%) was found in 14 (8%). Abnormal values were found for various laboratory tests with prolongation of platelet function analyzer-epinephrine (PFA-EPI) in 11%, PFA-ADP or arachidonic acid in 15.2%, PT in 35.9%, and APTT in 63.7%. Five-hundred-seventy-six patients (90%) had normal results in the coagulation factor assays and were categorized as patients with bleeding of unknown cause (BUC). A diagnosis of a bleeding disorder was more frequently made in men than in women (38 vs. 26). Iron deficiency anemia was found in 18 (25%) females positively associated with F-IX deficiency (p-value 0.000). Male gender (73.3%, p = 0.007) was independently associated with the diagnosis of coagulation factor deficiency.

Conclusion The current study reports a higher prevalence of coagulation factors deficiency in Saudi population than reported in the western population.

Authors' Contributions

K.A.S. and T.M.O. designed and developed the study. Both authors were responsible for contents and authenticity. N.A.-N., A.S., M.Z., A.A. oversaw data collection and data entry. N.A.O., E.A., N.B., and A.T. performed final review of data and analysis. F.A.Z., F.A.A., A.A.A., and R.S. were responsible for direction of the study team and facilitation of the project plan.


Note

The study was approved by the Institutional Review Board of King Faisal Specialist Hospital and Research Center, Kingdom of Saudi Arabia.




Publication History

Received: 18 August 2020

Accepted: 27 October 2020

Article published online:
23 December 2020

© 2020. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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  • References

  • 1 Peyvandi F, Palla R, Menegatti M, Mannucci PM. Introduction: rare bleeding disorders: general aspects of clinical features, diagnosis, and management. Semin Thromb Hemostasis 2009; 35 (04) 349-355
  • 2 Boender J, Kruip MJ, Leebeek FW. A diagnostic approach to mild bleeding disorders. J Thromb Haemost 2016; 14 (08) 1507-1516
  • 3 Greaves M, Watson HG. Approach to the diagnosis and management of mild bleeding disorders. J Thromb Haemost 2007; 5 (Suppl. 01) 167-174
  • 4 Hayward CP. Diagnosis and management of mild bleeding disorders. Hematology (Am Soc Hematol Educ Program) 2005; 2005 (01) 423-428
  • 5 Mezzano D, Quiroga T. Diagnostic challenges of inherited mild bleeding disorders: a bait for poorly explored clinical and basic research. J Thromb Haemost 2019; 17 (02) 257-270
  • 6 Gebhart J, Hofer S, Panzer S. et al. High proportion of patients with bleeding of unknown cause in persons with a mild-to-moderate bleeding tendency: results from the Vienna Bleeding Biobank (VIBB). Haemophilia 2018; 24 (03) 405-413
  • 7 Al-Fawaz IM, Gader AM, Bahakim HM, Al-Mohareb F, Al-Momen AK, Harakati MS. Hereditary bleeding disorders in Riyadh, Saudi Arabia. Ann Saudi Med 1996; 16 (03) 257-261
  • 8 El-Bostany EA, Omer N, Salama EE, El-Ghoroury EA, Al-Jaouni SK. The spectrum of inherited bleeding disorders in pediatrics. Blood Coagul Fibrinolysis 2008; 19 (08) 771-775
  • 9 Angiolillo D, Levy J, Schneider D, Jennings L. The Role of Platelet Function Testing in Improving Clinical Outcomes. The Medical Roundtable Cardiovascular Edition;. 2020
  • 10 Favaloro EJ. Clinical utility of the PFA-100. Semin Thromb Hemostasis 2008; 34: 709-733
  • 11 Paniccia R, Priora R, Liotta AA, Abbate R. Platelet function tests: a comparative review. Vasc Health Risk Manag 2015; 11: 133-148
  • 12 Owaidah T, Saleh M, Alzahrani H. et al. Prevalence of bleeding symptoms among adolescents and young adults in the capital city of Saudi Arabia. Adv Hematol 2018; 2018: 1858241
  • 13 Ahmed MA, Al-Sohaibani MO, Al-Mohaya SA, Sumer T, Al-Sheikh EH, Knox-Macaulay H. Inherited bleeding disorders in the Eastern Province of Saudi Arabia. Acta Haematol 1988; 79 (04) 202-206
  • 14 Al-Sharif FZ, Aljurf MD, Al-Momen AM. et al. Clinical and laboratory features of congenital factor XIII deficiency. Saudi Med J 2002; 23 (05) 552-554
  • 15 Hamamy H. Consanguineous marriages: preconception consultation in primary health care settings. J Community Genet 2012; 3 (03) 185-192
  • 16 Alharbi A, Hassan SB, Al-Momen A-K. et al. Influence of ABO blood group on von Willebrand factor tests in healthy Saudi blood donors. Blood Coagul Fibrinolysis 2018; 29 (02) 211-215
  • 17 Albanyan A, Al-Musa A, AlNounou R. et al. Diagnosis of Glanzmann thrombasthenia by whole blood impedance analyzer (MEA) vs. light transmission aggregometry. Int J Lab Hematol 2015; 37 (04) 503-508
  • 18 Sadler JE, Mannucci PM, Berntorp E. et al. Impact, diagnosis and treatment of von Willebrand disease. Thromb Haemost 2000; 84 (02) 160-174
  • 19 Madkhali I, Owaidah T, Riash MA, Naser R, Al-Zahrani H. Rare inherited clotting factor deficiencies: a single-center experience. J Appl Hematol 2012; 3 (01) 36-38
  • 20 Quiroga T, Goycoolea M, Panes O. et al. High prevalence of bleeders of unknown cause among patients with inherited mucocutaneous bleeding. A prospective study of 280 patients and 299 controls. Haematologica 2007; 92 (03) 357-365
  • 21 Friberg B, Ornö AK, Lindgren A, Lethagen S. Bleeding disorders among young women: a population-based prevalence study. Acta Obstet Gynecol Scand 2006; 85 (02) 200-206
  • 22 Ostrowsky J, Foes J, Warchol M, Tsarovsky G, Blay J. Plateletworks platelet function test compared to the thromboelastograph for prediction of postoperative outcomes. J Extra Corpor Technol 2004; 36 (02) 149-152
  • 23 Raman S, Silverman NA. Clinical utility of the platelet function analyzer (PFA-100) in cardiothoracic procedures involving extracorporeal circulation. J Thorac Cardiovasc Surg 2001; 122 (01) 190-191
  • 24 Fattorutto M, Pradier O, Schmartz D, Ickx B, Barvais L. Does the platelet function analyser (PFA-100) predict blood loss after cardiopulmonary bypass?. Br J Anaesth 2003; 90 (05) 692-693
  • 25 Forestier F, Coiffic A, Mouton C, Ekouevi D, Chêne G, Janvier G. Platelet function point-of-care tests in post-bypass cardiac surgery: are they relevant?. Br J Anaesth 2002; 89 (05) 715-721