Glanzmann's thrombasthenia classification: Difference between revisions

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==Overview==
==Overview==
Glycoprotein (GP)IIb-IIIa(aIIb,P3) is a Ca2+- G dependent heterodimer complex that belongs to the integrin family receptors involved in cell-cell and cell matrix adhesion. Normal platelets contain approximately 50,000 molecules of GPIIb-IIIa, which comprise 1% to 2% of the total platelet protein.<ref name="pmid1455408">{{cite journal| author=Kato A, Yamamoto K, Aoki N| title=Classification of Glanzmann's thrombasthenia based on the intracellular transport pathway of GPIIb-IIIa. | journal=Thromb Haemost | year= 1992 | volume= 68 | issue= 5 | pages= 615-6 | pmid=1455408 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1455408  }}</ref>


==Classification ==
==Classification ==
Glanzmann thrombasthenia (GT) is an autosomal recessive inherited qualitative platelet disorder characterized by absence or reduction of platelet glycoprotein GPIIb or GPIIIa. On the basic of levels of GPIIb/ IIIa as detected by CD61, GT has been subclassified into types I, II, and III. Patients with less than 5% of normal GPIIb/IIIa are classified as type I and patients with 5% to 20% normal GPIIb/IIIa are type II. Type III variants usually have dysfunctional receptors with near-normal GPIIb/ IIIa levels. Variability exists in the subtypes found in various ethnic groups. Type I GT is relatively frequent in Iraqi-Jews and Arabs residing in Israel, whereas type II GT is more often found in the Japanese population.<ref name="pmid14508803">{{cite journal| author=Kannan M, Ahmed RP, Jain P, Kumar R, Choudhry VP, Saxena R| title=Type I Glanzmann thrombasthenia: most common subtypes in North Indians. | journal=Am J Hematol | year= 2003 | volume= 74 | issue= 2 | pages= 139-41 | pmid=14508803 | doi=10.1002/ajh.10395 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14508803  }}</ref>


==References==
==References==

Revision as of 13:54, 2 July 2018

Glanzmann's thrombasthenia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Glycoprotein (GP)IIb-IIIa(aIIb,P3) is a Ca2+- G dependent heterodimer complex that belongs to the integrin family receptors involved in cell-cell and cell matrix adhesion. Normal platelets contain approximately 50,000 molecules of GPIIb-IIIa, which comprise 1% to 2% of the total platelet protein.[1]

Classification

Glanzmann thrombasthenia (GT) is an autosomal recessive inherited qualitative platelet disorder characterized by absence or reduction of platelet glycoprotein GPIIb or GPIIIa. On the basic of levels of GPIIb/ IIIa as detected by CD61, GT has been subclassified into types I, II, and III. Patients with less than 5% of normal GPIIb/IIIa are classified as type I and patients with 5% to 20% normal GPIIb/IIIa are type II. Type III variants usually have dysfunctional receptors with near-normal GPIIb/ IIIa levels. Variability exists in the subtypes found in various ethnic groups. Type I GT is relatively frequent in Iraqi-Jews and Arabs residing in Israel, whereas type II GT is more often found in the Japanese population.[2]

References

  1. Kato A, Yamamoto K, Aoki N (1992). "Classification of Glanzmann's thrombasthenia based on the intracellular transport pathway of GPIIb-IIIa". Thromb Haemost. 68 (5): 615–6. PMID 1455408.
  2. Kannan M, Ahmed RP, Jain P, Kumar R, Choudhry VP, Saxena R (2003). "Type I Glanzmann thrombasthenia: most common subtypes in North Indians". Am J Hematol. 74 (2): 139–41. doi:10.1002/ajh.10395. PMID 14508803.