Consanguineous marriage and rare bleeding disorders

Expert Rev Hematol. 2021 May;14(5):467-472. doi: 10.1080/17474086.2021.1923476. Epub 2021 May 10.

Abstract

Introduction: Rare bleeding disorders (RBDs) are a heterogeneous group of bleeding disorders. Consanguineous marriage is the favorite partnership in many communities among a population of more than a billion. Consanguineous marriages allow the defective recessive genes in RBDs to remain in the family. Clinical approach to RBDs is not as straightforward as typical bleeding disorders, which are secondary to scarcity and variation of RBDs. However, in many developing countries, the limitations of diagnostic facilities, the supply of therapeutic coagulation concentrates, health budgets, and optimal health-care infrastructures may intensify the clinical practice of RBDs.Areas covered: The author addresses the role of consanguineous marriage in the incidence of RBDs and what preventive steps can be considered to minimize the incidence of RBDs. A literature review of PubMed and google Scholar was conducted in November of 2020 using the relevant keywords.Expert opinion: This review addresses the prevalence of consanguineous marriages in Middle Eastern countries and the various reasons behind the coexistence of consanguineous marriage with RBDs. Finally, prevention steps that may be considered by health-care systems to minimize the incidence of rare bleeding disorders have been discussed. Health-care systems should train partners with the intention of consanguineous marriage on the downside of consanguinity.

Keywords: Rare bleeding disorders; consanguineous marriage; platelet disorders; von Willebrand disease (vWD).

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Blood Coagulation Disorders*
  • Consanguinity
  • Humans
  • Incidence
  • Prevalence
  • Rare Diseases / diagnosis
  • Rare Diseases / epidemiology
  • Rare Diseases / genetics