Glanzmann's thrombasthenia classification: Difference between revisions
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{{Glanzmann's thrombasthenia}} | {{Glanzmann's thrombasthenia}} | ||
{{CMG}} | {{CMG}} {{AE}} {{OK}}, [[User:Niush.D|Niyousha Danesh, MD-MPH]] | ||
==Overview== | ==Overview== | ||
[[Glanzmann's thrombasthenia]] is mainly divided into hereditary GT, variant GT, and acquired GT. [[Glanzmann's thrombasthenia|Glanzmann thrombasthenia]] (GT) is an [[autosomal recessive]] inherited qualitative [[platelet disorder]] characterized by absence or reduction of [[platelet]] [[glycoprotein]] [[Glycoprotein IIb/IIIa|GPIIb]] or GPIIIa or [[CD61]]. [[Hereditary]] [[Glanzmann's thrombasthenia|Glanzmann thrombasthenia]] is classified into three types The subtypes vary based on ethnicity. For example, Type I is more common in Arabs and Iraqi-Jews living in Israel, whilst type II is relatively frequent in the Japanese population. Variant type includes patients with [[Platelet|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 reason being that the stimulated [[platelets]] can not bind to soluble Fg or [[antibodies]] recognizing activation-dependent determinants on [[αIIbβ3]]. It is due to a single amino acid substitution. Acquired GT is defined by [[inhibition]] of [[platelet]] αIIbβ3 actual function due to the attack of [[autoantibodies]]. These [[antibodies]] can be produced in numerous disorders such as [[hematologic]] [[malignancy]], [[transfusion]], [[drugs]] and [[autoimmune diseases]][[autoimmune diseases.|.]] | |||
==Classification == | ==Classification == | ||
== | [[Glanzmann's thrombasthenia]] is mainly divided into the following types:<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><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><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> | ||
Glanzmann thrombasthenia | |||
===Hereditary GT=== | |||
[[Glanzmann's thrombasthenia|Glanzmann thrombasthenia]] (GT) is an [[autosomal recessive]] inherited qualitative [[platelet disorder]] characterized by absence or reduction of [[platelet]] [[glycoprotein]] [[Glycoprotein IIb/IIIa|GPIIb]] or GPIIIa or [[CD61]]. Glanzmann thrombasthenia is classified into three types | |||
* Patients with less than 5% of normal [[Glycoprotein IIb/IIIa|GPIIb/IIIa]] are classified as type I | |||
* Type II variants have 5% to 20% normal [[Glycoprotein IIb/IIIa|GPIIb/IIIa]] | |||
* ype III possess near-normal GPIIb/ IIIa levels but dysfunctional receptors | |||
The subtypes vary based on ethnicity. For example, Type I is more common in Arabs and Iraqi-Jews living in Israel, whilst type II 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> | |||
=== Variant GT === | |||
This subset includes patients with [[Platelet|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 reason being that the stimulated [[platelets]] can not bind to soluble Fg or [[antibodies]] recognizing activation-dependent determinants on [[αIIbβ3]]. It is due to a single amino acid substitution. <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 defined by inhibition of platelet αIIbβ3 actual function due to the attack of [[autoantibodies]]. These antibodies can be produced in numerous disorders such as hematologic malignancy, transfusion, drugs and [[autoimmune diseases.]]<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]]. These [[antibodies]] can be produced in numerous disorders such as [[hematologic]] [[malignancy]], [[transfusion]], [[drugs]] and [[autoimmune diseases]][[autoimmune diseases.|.]]<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== | ||
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[[Category:Disease]] | [[Category:Disease]] | ||
[[Category:Hematology]] | [[Category:Hematology]] | ||
Latest revision as of 21:52, 29 July 2020
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Glanzmann's thrombasthenia |
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Differentiating Glanzmann's thrombasthenia from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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Glanzmann's thrombasthenia classification On the Web |
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American Roentgen Ray Society Images of Glanzmann's thrombasthenia classification |
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Risk calculators and risk factors for Glanzmann's thrombasthenia classification |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Omer Kamal, M.D.[2], Niyousha Danesh, MD-MPH
Overview
Glanzmann's thrombasthenia is mainly divided into hereditary GT, variant GT, and acquired GT. Glanzmann thrombasthenia (GT) is an autosomal recessive inherited qualitative platelet disorder characterized by absence or reduction of platelet glycoprotein GPIIb or GPIIIa or CD61. Hereditary Glanzmann thrombasthenia is classified into three types The subtypes vary based on ethnicity. For example, Type I is more common in Arabs and Iraqi-Jews living in Israel, whilst type II is relatively frequent in the Japanese population. Variant type 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 reason being that the stimulated platelets can not bind to soluble Fg or antibodies recognizing activation-dependent determinants on αIIbβ3. It is due to a single amino acid substitution. Acquired GT is defined by inhibition of platelet αIIbβ3 actual function due to the attack of autoantibodies. These antibodies can be produced in numerous disorders such as hematologic malignancy, transfusion, drugs and autoimmune diseases.
Classification
Glanzmann's thrombasthenia is mainly divided into the following types:[1][2][3]
Hereditary GT
Glanzmann thrombasthenia (GT) is an autosomal recessive inherited qualitative platelet disorder characterized by absence or reduction of platelet glycoprotein GPIIb or GPIIIa or 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
- ype III possess near-normal GPIIb/ IIIa levels but dysfunctional receptors
The subtypes vary based on ethnicity. For example, Type I is more common in Arabs and Iraqi-Jews living in Israel, whilst type II is relatively frequent in the Japanese population.[1]
Variant GT
This subset 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 reason being that the stimulated platelets can not bind to soluble Fg or antibodies recognizing activation-dependent determinants on αIIbβ3. It is due to a single amino acid substitution. [2]
Acquired GT
Acquired GT is defined by inhibition of platelet αIIbβ3 actual function due to the attack of autoantibodies. These antibodies can be produced in numerous disorders such as hematologic malignancy, transfusion, drugs and autoimmune diseases.[3]
References
- ↑ 1.0 1.1 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.
- ↑ 2.0 2.1 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.
- ↑ 3.0 3.1 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).