Glanzmann's thrombasthenia historical perspective
|
Glanzmann's thrombasthenia |
|
Differentiating Glanzmann's thrombasthenia from other Diseases |
|---|
|
Diagnosis |
|
Treatment |
|
Case Studies |
|
Glanzmann's thrombasthenia historical perspective On the Web |
|
American Roentgen Ray Society Images of Glanzmann's thrombasthenia historical perspective |
|
Glanzmann's thrombasthenia historical perspective in the news |
|
Risk calculators and risk factors for Glanzmann's thrombasthenia historical perspective |
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]
In 1966, Caen et al.2 explained15 patients with Glanzmann’s thrombasthenia, with decreased or nil platelet aggregation but the clot retraction was sometimes only mildly effected.[2]
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 2.2 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.