Glanzmann's thrombasthenia epidemiology and demographics

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Glanzmann's thrombasthenia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Niyousha Danesh, MD-MPH

Overview

Epidemiology and Demographics

  • The incidence/prevalence of Glanzmann's thrombasthenia is approximately one per 1,000,000 individuals worldwide.[1]
  • The highest reported prevalence in the world was in Iran, in 2004 the incidence of Glanzmann's thrombasthenia was approximately 2 per 100,000 individuals.[2]
  •  84% of patients with Glanzmann's thrombasthenia necessitate red blood cell transfusion at least once in their lifespan.
  • Fatal bleeding can occur at any age in GT patients, however the prevalence of severe bleeding episodes are reduce with age.
  • The case-fatality rate of Glanzmann's thrombasthenia is relatively low.
  • Age:
    • The incidence of Glanzmann's thrombasthenia decreases with age; the median age at diagnosis is 8 years.[2]
    • Glanzmann's thrombasthenia commonly manifests in individuals younger than 5 years of age.
  • Race:
    • Glanzmann's thrombasthenia usually affects individuals of the Arabs,Southern Indians,Hindus,Jordanians, Iranians,Palestinian, Iraqi Jews and French Gypsies races. individuals of the other parts of the world are less likely to develop GT.[3][4]
    • Twenty two patients (12 males and 10 females) from all over Israel fulfilled the ‘Criteria for establishing the diagnosis of Glanzmann’s thrombasthenia.[5]
  • Sex:
    • Women are slightly more affected by Glanzmann's thrombasthenia  than men. The female to male ratio is approximately 1.38 to 1.
    • The majority of Glanzmann's thrombasthenia cases are reported in middle east including individuals of Palestinian, Israel Iran, Iraq, Saudi Arabia,India, Jordan and France. while in these ethnic groups consanguineous marriages is more prevalent. [6]

References

  1. Solh T, Botsford A, Solh M (2015). "Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options". J Blood Med. 6: 219–27. doi:10.2147/JBM.S71319. PMC 4501245. PMID 26185478.
  2. 2.0 2.1 Toogeh G, Sharifian R, Lak M, Safaee R, Artoni A, Peyvandi F (2004). "Presentation and pattern of symptoms in 382 patients with Glanzmann thrombasthenia in Iran". Am J Hematol. 77 (2): 198–9. doi:10.1002/ajh.20159. PMID 15389911.
  3. Coller BS, Seligsohn U, Zivelin A, Zwang E, Lusky A, Modan M (April 1986). "Immunologic and biochemical characterization of homozygous and heterozygous Glanzmann thrombasthenia in the Iraqi-Jewish and Arab populations of Israel: comparison of techniques for carrier detection". Br. J. Haematol. 62 (4): 723–35. PMID 2938617.
  4. Jacquelin B, Tuleja E, Kunicki TJ, Nurden P, Nurden AT (March 2003). "Analysis of platelet membrane glycoprotein polymorphisms in Glanzmann thrombasthenia showed the French gypsy mutation in the alphaIIb gene to be strongly linked to the HPA-1b polymorphism in beta3". J. Thromb. Haemost. 1 (3): 573–5. PMID 12871468.
  5. Reichert N, Seligsohn U, Ramot B (December 1975). "Clinical and genetic aspects of Glanzmann's thrombasthenia in Israel: report of 22 cases". Thromb Diath Haemorrh. 34 (3): 806–20. PMID 1239828.
  6. Solh T, Botsford A, Solh M (2015). "Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options". J Blood Med. 6: 219–27. doi:10.2147/JBM.S71319. PMC 4501245. PMID 26185478.