Glanzmann's thrombasthenia overview
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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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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Niyousha Danesh, M.D., MPH.
Overview
Glanzmann's thrombasthenia is an extremely rare disorder of the blood, in which the platelets lack glycoprotein IIb/IIIa. Hence, no fibrinogen bridging can occur, and bleeding time is significantly prolonged.
Historical Perspective
In 1918, Eduard Glanzmann, a Swiss pediatrician, first described this disease.[1]
In 1956, Braunsteiner and Pakesch reviewed disorders of platelet function and described thrombasthenia as an inherited disease characterized by platelets of normal size that failed to spread onto the surface and did not support clot retraction.
In 1964 the diagnostic features of GT, including the absence of platelet aggregation as the primary feature were clearly established by the classic report on 15 French patients by Caen et al.
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]
Classification
Pathophysiology
Causes
Differentiating Glanzmann's thrombasthenia overview from Other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications, and Prognosis
Natural History
Complications
Prognosis
Diagnosis
Diagnostic Criteria
History and Symptoms
Physical Examination
Laboratory Findings
Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Prevention
References
- ↑ 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.
- ↑ Nurden AT (2006). "Glanzmann thrombasthenia". Orphanet J Rare Dis. 1: 10. doi:10.1186/1750-1172-1-10. PMC 1475837. PMID 16722529.