Glanzmann's thrombasthenia classification: Difference between revisions
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==Overview== | ==Overview== | ||
Revision as of 16:29, 13 November 2018
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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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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
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] GT is classified in to two types : hereditary GT, which is sub classified into 3 types,( type I
, II and III ) and acquired GT.
Classification
Hereditary 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. These antibodies can be produced in numerous disorders such as hematologic malignancy, transfusion, drugs and autoimmune diseases.[6]
References
- ↑ 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.
- ↑ 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.
- ↑ 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). - ↑ 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.
- ↑ 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.
- ↑ 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).