thrombasthenia

(redirected from Glanzmann's thrombasthenia)
Also found in: Thesaurus, Medical, Wikipedia.
Related to Glanzmann's thrombasthenia: Bernard-Soulier syndrome, Von Willebrand disease
ThesaurusAntonymsRelated WordsSynonymsLegend:
Noun1.thrombasthenia - a rare autosomal recessive disease in which the platelets do not produce clots in the normal way and hemorrhage results
autosomal recessive defect, autosomal recessive disease - a disease caused by the presence of two recessive mutant genes on an autosome
References in periodicals archive ?
Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options.
Hurford, "Glanzmann's thrombasthenia: report of a case and review of the literature," International Journal of Clinical and Experimental Pathology, vol.
Hematological and surgical management in Glanzmann's thrombasthenia: A case report.
Platelet function disorders vary in severity, with some of the most severe including Glanzmann's thrombasthenia (GT) and Bernard-Soulier syndrome; however, other conditions which are generally milder may also occasionally produce severe bleeding symptoms.
Exclusion criteria: Patients with diabetes, platelet adhesion defects such as von Willebrand disease, Bernard-Soulier syndrome or platelet aggregation defects such as Glanzmann's thrombasthenia, afibrinogenemia; history of an infectious disease in the last 10 days and use of at least one of following drugs in the last two weeks: acetylsalicylic acid, clopidogrel, ticlopidine, beta lactam antibiotics, corticosteroids and non-steroidal anti-inflammatory drugs.
Glanzmann's thrombasthenia is an inherited haemorrhagic disorder characterized by a severe reduction in or absence of platelet aggregation in response to multiple physiologic agonists due to qualitative or quantitative abnormalities of platelet glycoprotein IIb-IIIa.
Vesical injury, chronic vesical irritation, bladder surgery, food and drug allergies, tuberculosis and malignancies can be causal factors of EC; however, etiologic agents cannot easily be identified.[sup.4] Intravesical mitomycin, thiotepa and dimethyl sulfoxide have been reported as possible causative agents.[sup.6,7] Food allergens and parasites, such as Toxocara canis and Echinococcus, have been used to clarify the onset of EC.[sup.8] Conditions that have been associated with EC include Glanzmann's thrombasthenia, eosinophilic gastroenteritis, bronchial asthma, and coeliac disease.[sup.9] Although peripheral eosinophilia and eosinophilia were highly suggestive of an underlying allergic cause, no specific cause was identified in this study.
Glanzmann's Thrombasthenia is a hereditary platelet dysfunction due to quantitative or qualitative defect in the platelet glycoprotein [alpha]IIb/[beta]3 the major integrin complex which leads to inability of plate aggregation by attachment to fibrinogen, leading to non -formation of platelet plug, and thus excessive, apparently spontaneous bleeding.
The facility's goal is to develop and maintain standardized iPSCs lines specific to a variety of rare inherited diseases--not only DBA and JMML, but also dyskeratosis congenita, congenital dyserythropoietic anemia, thrombocytopenia absent radu (TAR), Glanzmann's thrombasthenia and Hermansky-Pudlak syndrome.
Criteria for diagnosis of Glanzmann's thrombasthenia (GT) were normal PT, aPTT, platelet count, and morphology; no curve with ADP, epinephrine, or collagen; and normal curve with ristocetin in platelet aggregation by lumiaggregometry.