Glanzmann's thrombasthenia historical perspective
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Glanzmann's thrombasthenia |
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Differentiating Glanzmann's thrombasthenia from other Diseases |
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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
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. Later, it was defined as a heritable platelet disorder secondary to a dysfunction in GPIIb/IIIa complex. In 1956, Braunsteiner and Pakesch described Glanzmann's thrombasthenia as an inherited disorder with normal sized platelets that failed clot retraction. 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 1966, Caen et al. explained 15 patients with Glanzmann's thrombasthenia, with decreased or nil platelet aggregation but the clot retraction was sometimes only mildly effected. The variant disease was first established in 1987 In the mid 1970’s, Nurden and Caen and Phillips et al. discovered a deficiency of both GPIIb/GPIIIa in thrombasthenic platelets. Today, it 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). Glanzmann's thrombasthenia served as a template for platelet aggregation process as well as targets for therapeutic measures.
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 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 a deficiency of both GPIIb/GPIIIa in thrombasthenic platelets.[3]
In 1966, Caen et al. explained 15 patients with Glanzmann's thrombasthenia, with decreased or nil platelet aggregation but the clot retraction was sometimes only mildly effected.[2]The variant disease was first established in 1987.[2]
Today, it 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). Glanzmann's thrombasthenia served as a template for platelet aggregation process 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.