Glanzmann's thrombasthenia historical perspective: Difference between revisions
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In 1918, Eduard Glanzmann, a Swiss [[pediatrician]], described [[Glanzmann's thrombasthenia]] for the first time. It was known formerly as “[[hereditary]] [[hemorrhagic]] [[thrombasthenia]]”, but Glanzmann proposed it was not abnormal [[platelet]] number but a disorder of [[clotting]]<ref name="pmid26185478">{{cite journal |vauthors=Solh T, Botsford A, Solh M |title=Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options |journal=J Blood Med |volume=6 |issue= |pages=219–27 |date=2015 |pmid=26185478 |pmc=4501245 |doi=10.2147/JBM.S71319 |url=}}</ref>. Later, it was defined as a heritable platelet disorder secondary to a dysfunction in [[GPIIb/IIIa]] complex.<ref name="pmid26185478">{{cite journal| author=Solh T, Botsford A, Solh M| title=Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options. | journal=J Blood Med | year= 2015 | volume= 6 | issue= | pages= 219-27 | pmid=26185478 | doi=10.2147/JBM.S71319 | pmc=4501245 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26185478 }}</ref> | In 1918, Eduard Glanzmann, a Swiss [[pediatrician]], described [[Glanzmann's thrombasthenia]] for the first time. It was known formerly as “[[hereditary]] [[hemorrhagic]] [[thrombasthenia]]”, but Glanzmann proposed it was not abnormal [[platelet]] number but a disorder of [[clotting]]<ref name="pmid26185478">{{cite journal |vauthors=Solh T, Botsford A, Solh M |title=Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options |journal=J Blood Med |volume=6 |issue= |pages=219–27 |date=2015 |pmid=26185478 |pmc=4501245 |doi=10.2147/JBM.S71319 |url=}}</ref>. Later, it was defined as a heritable platelet disorder secondary to a dysfunction in [[GPIIb/IIIa]] complex.<ref name="pmid26185478">{{cite journal| author=Solh T, Botsford A, Solh M| title=Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options. | journal=J Blood Med | year= 2015 | volume= 6 | issue= | pages= 219-27 | pmid=26185478 | doi=10.2147/JBM.S71319 | pmc=4501245 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26185478 }}</ref> | ||
In 1956, Braunsteiner and Pakesch described [[Glanzmann's thrombasthenia]] as an inherited disorder with normal sized platelets that failed clot retraction. | In 1956, Braunsteiner and Pakesch described [[Glanzmann's thrombasthenia]] as an inherited disorder with normal sized platelets that failed clot retraction.<ref name="pmid16722529">{{cite journal |vauthors=Nurden AT |title=Glanzmann thrombasthenia |journal=Orphanet J Rare Dis |volume=1 |issue= |pages=10 |date=April 2006 |pmid=16722529 |pmc=1475837 |doi=10.1186/1750-1172-1-10 |url=}}</ref> | ||
About 50 years later, the development of methods for studying platelets demonstrated that thrombasthenic patients failed to aggregate in response to physiological agonists such as [[Adenosine diphosphate|ADP]], [[epinephrine]], [[collagen]] and [[thrombin]] had markedly reduced levels of platelet [[fibrinogen]] and had reduced or absent clot retraction. | About 50 years later, the development of methods for studying platelets demonstrated that thrombasthenic patients failed to aggregate in response to physiological agonists such as [[Adenosine diphosphate|ADP]], [[epinephrine]], [[collagen]] and [[thrombin]] had markedly reduced levels of platelet [[fibrinogen]] and had reduced or absent clot retraction. | ||
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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
Historical Perspective
In 1918, Eduard Glanzmann, a Swiss pediatrician, described Glanzmann's thrombasthenia for the first time. It was known formerly as “hereditary hemorrhagic thrombasthenia”, but Glanzmann proposed it was not abnormal platelet number but a disorder of clotting[1]. Later, it was defined as a heritable platelet disorder secondary to a dysfunction in GPIIb/IIIa complex.[1]
In 1956, Braunsteiner and Pakesch described Glanzmann's thrombasthenia as an inherited disorder with normal sized platelets that failed clot retraction.[2]
About 50 years later, the development of methods for studying platelets demonstrated that thrombasthenic patients failed to aggregate in response to physiological agonists such as ADP, epinephrine, collagen and thrombin had markedly reduced levels of platelet fibrinogen and had reduced or absent clot retraction.
In 1966, Caen et al.2 described 15 patients with Glanzmann’s thrombasthenia in which platelet aggregation was either nil or drastically decreased but the clot retraction was sometimes only slightly diminished.
In 1965, Castaldi and Caen 7 showed that the platelet fibrinogen was either strongly diminished (in parallel with the impaired clot retraction) or borderline to the normal range.
In the mid 1970’s Nurden and Caen and Phillips et al. discovered that thrombasthenic platelets were deficient in both GPIIb and GPIIIa.[3]
Those patients with absent platelet aggregation and absent clot retraction were subsequently termed as having type I disease; those with absent aggregation but residual clot retraction, type II disease; while variant disease was first established in 1987.[2]
Today GT receives much recognition, as it was one of the first disorders to define GPIIb/IIIa as a platelet receptor for adhesive molecules (such as VWF and fibrinogen). The disease also served as a template for understanding processes of platelet aggregation as well as targets for therapeutic measures.[1]
References
- ↑ 1.0 1.1 1.2 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.
- ↑ 2.0 2.1 Nurden AT (April 2006). "Glanzmann thrombasthenia". Orphanet J Rare Dis. 1: 10. doi:10.1186/1750-1172-1-10. PMC 1475837. PMID 16722529.
- ↑ Nair S, Ghosh K, Kulkarni B, Shetty S, Mohanty D (2002). "Glanzmann's thrombasthenia: updated". Platelets. 13 (7): 387–93. doi:10.1080/0953710021000024394. PMID 12487785.