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  • noun

Words related to thrombasthenia

a rare autosomal recessive disease in which the platelets do not produce clots in the normal way and hemorrhage results

References in periodicals archive ?
(12) Platelet aggregation can diagnose a variety of inherited platelet disorders including abnormalities of the GP1b-V-IX complex (Bernard Soulier syndrome), GPIIbIIIa (Glanzmann thrombasthenia), ADP receptor, thromboxane pathway, and secretion pathways.
Gianni, "Oral surgery in patients with Glanzmann thrombasthenia: a case series," Journal of Oral and Maxillofacial Surgery, vol.
A rare case report on Glanzmann thrombasthenia. Natl J Physiol Pharm Pharmacol 2017;7(11):1291-1292.
Peyvandi, "Presentation and pattern of symptoms in 382 patients with Glanzmann thrombasthenia in Iran," American Journal of Hematology, vol.
A systematic review of the management and outcomes of pregnancy in glanzmann thrombasthenia. Haemophilia 2011;17(5):858-69.
Glanzmann Thrombasthenia is a genetic platelet disorder in which platelet glycoprotein [alpha]IIb/[beta]3 the major integrin complex (fibrinogen receptor) is either deficient or present but dysfunctional.
In 20% (n=12) of the patients, a bleeding disorder was detected (1 case of type 3 von Willebrand disease, 2 patients with low VWF:Ag, 1 case of probable von Willebrand disease, 3 cases of Bernard-Soulier syndrome, 2 cases of Glanzmann thrombasthenia, 2 cases of immune thrombocytopenic purpura, 1 case of congenital factor VII deficiency).
Glanzmann thrombasthenia (GT) is caused by an abnormality in the genes which encode glycoproteins IIb/IIIa, the glycoprotein Ilb/IIIa receptor (also called the fibrinogen receptor).
Hemarthrosis is rarely seen in disorders of platelet function and occurs even more rarely in Glanzmann thrombasthenia (GT), whereas episodes of hemarthrosis can be frequent in hemophilia (1).
Glanzmann thrombasthenia is the diagnosis which should be considered primarily in patients with normal platelet count and unusual cutaneous and mucosal bleedings starting from birth and early childhood.
Glanzmann thrombasthenia, a membrane defect characterized by dysfunction or loss of the GP IIb/IIIa receptor site, may be diagnosed by its characteristically diminished secretion and aggregation responses to all agonists with the exception of a modest response to arachidonic acid.
For example, Glanzmann thrombasthenia is a deficiency of the IIb and/or IIIa subunits of the GP IIb/IIIa receptor.