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These subtypes vary based on ethnicity . For example Type I GT is found commonly in Arabs and Iraqi-Jews living in Israel, whilst type II GT is relatively frequent 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>
These subtypes vary based on ethnicity . For example Type I GT is found commonly in Arabs and Iraqi-Jews living in Israel, whilst type II GT is relatively frequent 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>


GT has also another subtype named as Variant GT, this group consists of patients with platelets expression of αIIbβ3 more than 20% which normally support platelet aggregation but they present the clinical phenotype of GT . variants are characterized by the inability of stimulated platelets to bind soluble Fg or antibodies recognizing activation-dependent determinants on αIIbβ3. They are mostly given by single amino acid substitutions with the nature and position of the affected residue defining the residual functional response. As for the alloantigen systems carried by αIIbβ3, most variant forms concern ''ITGB3''.
GT has also another subtype named as Variant GT, this group includes patients with platelets expression of αIIbβ3 more than 20% in which mainly the platelets are able to aggregate but they present the clinical phenotype of GT. The principal reason is  that the stimulated platelets can not bind to soluble Fg or antibodies recognizing activation-dependent determinants on αIIbβ3. T It is commonly due to substitutions in single amino acid . <ref name="pmid23929305">{{cite journal| author=Nurden AT, Pillois X, Wilcox DA| title=Glanzmann thrombasthenia: state of the art and future directions. | journal=Semin Thromb Hemost | year= 2013 | volume= 39 | issue= 6 | pages= 642-55 | pmid=23929305 | doi=10.1055/s-0033-1353393 | pmc=4011384 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23929305  }}</ref>


== Acquired GT ==
== Acquired GT ==
Acquired GT is commonly the result of an autoantibody attack on platelet αIIbβ3, or iso antibodies inhibiting proper function. The production of autoantibodies has been associated with platelet transfusions, as well as numerous hematologic conditions, including immune thrombocytopenic purpura, non-Hodgkin’s lymphoma, multiple myeloma, myelodysplastic syndrome, hairy cell leukemia, and acute lymphoblastic leukemia. In a case report done by Blickstein et al, a patient with systemic lupus erythematosus presented in adulthood with degrees of mucocutaneous bleeding secondary to antibody production against GPIIb/IIIa.5 Also, some anti-thrombotic therapies use αIIbβ3 antagonists, such as abciximab, eptifibatide, and tirofiban, for treatment of acute coronary events, which can trigger a transient GT-like state.<ref name="pmid1990;75:1383–952">{{cite journal| author=Arimura H| title=Correlation between molecular size and interferon- inducing activity of poly I:C. | journal=Acta Virol | year= 1975 | volume= 19 | issue= 6 | pages= 457-66 | pmid=1990;75:1383–95 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1990  }}</ref>
Acquired GT is defined by inhibition of platelet αIIbβ3 actual function due to the attack of autoantibodies. The production of autoantibodies has been associated with platelet transfusions, as well as numerous hematologic conditions, including immune thrombocytopenic purpura, non-Hodgkin’s lymphoma, multiple myeloma, myelodysplastic syndrome, hairy cell leukemia, and acute lymphoblastic leukemia. In a case report done by Blickstein et al, a patient with systemic lupus erythematosus presented in adulthood with degrees of mucocutaneous bleeding secondary to antibody production against GPIIb/IIIa.5 Also, some anti-thrombotic therapies use αIIbβ3 antagonists, such as abciximab, eptifibatide, and tirofiban, for treatment of acute coronary events, which can trigger a transient GT-like state.<ref name="pmid1990;75:1383–952">{{cite journal| author=Arimura H| title=Correlation between molecular size and interferon- inducing activity of poly I:C. | journal=Acta Virol | year= 1975 | volume= 19 | issue= 6 | pages= 457-66 | pmid=1990;75:1383–95 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1990  }}</ref>


==References==
==References==

Revision as of 14:59, 6 July 2018

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

Overview

Glycoprotein (GP)IIb-IIIa(αIIbβ3) 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] The two genes, encoding for GPIIb (ITGA2B) and GPIIIa (ITGB3) are closely associated at chromosome 17q21.[2]

Classification

Inherent GT

Glanzmann thrombasthenia (GT) is an autosomal recessive inherited qualitative platelet disorder characterized by absence or reduction of platelet glycoprotein GPIIb or GPIIIa. CD61, Glanzmann thrombasthenia is classified into three types :

Patients with less than 5% of normal GPIIb/IIIa are classified as type I

Type II variants have 5% to 20% normal GPIIb/IIIa .

And Type III patients possess near-normal GPIIb/ IIIa levels but dysfunctional receptors.

these classification is according to clot retraction and platelet fibrinogen content [3]

These subtypes vary based on ethnicity . For example Type I GT is found commonly in Arabs and Iraqi-Jews living in Israel, whilst type II GT is relatively frequent in the Japanese population.[4]

GT has also another subtype named as Variant GT, this group includes patients with platelets expression of αIIbβ3 more than 20% in which mainly the platelets are able to aggregate but they present the clinical phenotype of GT. The principal reason is that the stimulated platelets can not bind to soluble Fg or antibodies recognizing activation-dependent determinants on αIIbβ3. T It is commonly due to substitutions in single amino acid . [5]

Acquired GT

Acquired GT is defined by inhibition of platelet αIIbβ3 actual function due to the attack of autoantibodies. The production of autoantibodies has been associated with platelet transfusions, as well as numerous hematologic conditions, including immune thrombocytopenic purpura, non-Hodgkin’s lymphoma, multiple myeloma, myelodysplastic syndrome, hairy cell leukemia, and acute lymphoblastic leukemia. In a case report done by Blickstein et al, a patient with systemic lupus erythematosus presented in adulthood with degrees of mucocutaneous bleeding secondary to antibody production against GPIIb/IIIa.5 Also, some anti-thrombotic therapies use αIIbβ3 antagonists, such as abciximab, eptifibatide, and tirofiban, for treatment of acute coronary events, which can trigger a transient GT-like state.[6]

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. Manne RK, Natarajan K, Patil R, Prathi VS, Beeraka SS, Kolaparthi VS (2014). "Glanzmann thrombasthenia associated with human immunodeficiency virus-positive patient". Int J Prev Med. 5 (4): 500–4. PMC 4018600. PMID 24829739.
  3. Arimura H (1975). "Correlation between molecular size and interferon- inducing activity of poly I:C". Acta Virol. 19 (6): 457–66. PMID 1990;75:1383–95 Check |pmid= value (help).
  4. 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.
  5. Nurden AT, Pillois X, Wilcox DA (2013). "Glanzmann thrombasthenia: state of the art and future directions". Semin Thromb Hemost. 39 (6): 642–55. doi:10.1055/s-0033-1353393. PMC 4011384. PMID 23929305.
  6. Arimura H (1975). "Correlation between molecular size and interferon- inducing activity of poly I:C". Acta Virol. 19 (6): 457–66. PMID 1990;75:1383–95 Check |pmid= value (help).