Glanzmann's thrombasthenia overview: Difference between revisions

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{{Glanzmann's thrombasthenia}}
{{Glanzmann's thrombasthenia}}


{{CMG}}
{{CMG}} '''Associate Editor(s)-in-Chief: [[Niyousha Danesh, M.D., MPH.]]'''


==Overview==
==Overview==
'''Glanzmann's thrombasthenia''' is an extremely rare [[Blood diseases|disorder]] of the [[blood]], in which the [[platelet]]s lack [[glycoprotein IIb/IIIa]]. Hence, no [[fibrinogen]] bridging can occur, and bleeding time is significantly prolonged.
'''Glanzmann's thrombasthenia''' is an extremely rare [[Blood diseases|disorder]] of the [[blood]], in which the [[platelet]]s lack [[glycoprotein IIb/IIIa]]. Hence, no [[fibrinogen]] bridging can occur, and bleeding time is significantly prolonged.  


==Historical Perspective==
==Historical Perspective==

Revision as of 05:29, 2 July 2018

Glanzmann's thrombasthenia

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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

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History and Symptoms

Physical Examination

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

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References

  1. 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. Nurden AT (2006). "Glanzmann thrombasthenia". Orphanet J Rare Dis. 1: 10. doi:10.1186/1750-1172-1-10. PMC 1475837. PMID 16722529.

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