Glanzmann thrombasthenia


Also found in: Dictionary, Thesaurus.

thrombasthenia

 [throm″bas-the´ne-ah]
1. decreased platelet function; called also thromboasthenia.
Glanzmann thrombasthenia a hereditary platelet abnormality characterized by defective clot retraction, prolonged bleeding time, and related symptoms such as epistaxis and inappropriate bleeding. Clinically there is abnormal glass adhesion and impaired aggregation to ADP, collagen, and thrombin. Called also thrombasthenia and Glanzmann's disease.

Glanz·mann throm·bas·the·ni·a

(glahntz'mahn), [MIM*187800]
a hemorrhagic diathesis characterized by normal or prolonged bleeding time, normal coagulation time, defective clot retraction, and normal platelet count, but morphologic or functional abnormality of platelets; several different kinds of platelet abnormalities have been described; caused by defect in platelet membrane glycoprotein IIb-IIIa complex; autosomal recessive inheritance, caused by mutation in the platelet-membrane glycoprotein IIb-IIIa complex gene (ITGA2B) on chromosome 17.

Glanz·mann thromb·as·the·ni·a

(glahnts'mahn throm'bas-thē'nē-ă)
A hemorrhagic diathesis characterized by normal or prolonged bleeding time, normal coagulation time, defective clot retraction, and normal platelet count but morphologic or functional abnormality of platelets.

Glanzmann,

Eduard, Swiss clinician, 1887-1959.
Glanzmann disease - Synonym(s): Glanzmann thrombasthenia
Glanzmann-Naegeli syndrome - Synonym(s): Glanzmann thrombasthenia
Glanzmann and Riniker lymphocytophthisis - agammaglobulinemia, absent thymus, severe cytopenia, recurring infections, and inability to form antibodies.
Glanzmann thrombasthenia - a hemorrhagic diathesis due to defect in platelet membrane glycoprotein IIb-IIIa complex. Synonym(s): constitutional thrombopathy; Glanzmann disease; Glanzmann-Naegeli syndrome; hereditary hemorrhagic thrombasthenia
References in periodicals archive ?
Nandakumar, "Glanzmann thrombasthenia: a rare hematological disorder with oral manifestations: a case report," The Journal of Contemporary Dental Practice, vol.
As Comparative to a case of Von-Willebrand Disease the prognosis of Glanzmann Thrombasthenia improves with age.
Dx: Diagnosis, Fibr: Fibrinogen, FVII def.: FVII defciency, * FVII leve was 20%, ND: Not done, VWD: Von Willebrand disease, VWF: Von Wolebrand factor, BSS: Bernard-Soulier syndrome, GT: Glanzmann thrombasthenia In patients with Needing disorders, a history of mucosal bleeding, hypoferritinemia, and prolonged closure time were significantly more frequent (p<0.05).
Defects in Glanzmann thrombasthenia and LAD-III (LAD-1/v) syndrome: the role of integrin [beta]1 and [beta]3 in platelet adhesion to collagen.
[3] Nonstandard abbreviations: RI, reference interval; GPIIb/IIIa, glycoprotein IIb/IIIa; GT, Glanzmann thrombasthenia; LTA, light transmission aggregometry; PRP, platelet-rich plasma.
Since Glanzmann thrombasthenia is a disease caused by platelet dysfunction, there is defect in primary clot formation and mucosal bleedings are observed frequently.
Molecular study of Glanzmann thrombasthenia in 3 patients issued from 2 different families.
describe the defect in each of the following hereditary disorders: Glanzmann thrombasthenia and Bernard-Soulier syndrome (BSS).
In von Willebrand disease, Glanzmann thrombasthenia, and myeloproliferative disorders, the platelets have typical morphologic features, whereas giant platelets are seen in Bernard-Soulier disease and other macrothrombocytopenia syndromes.
The pathogenesis and molecular defects of many primary thrombocytopathies are well known and relate to defects in structural or functional glycoproteins, such as the abnormal expression of gpIIb/IIIa in Glanzmann thrombasthenia and gpIb in Bernard-Soulier disease (8994).
Patients with afibrinogenemia or Glanzmann thrombasthenia (abnormalities of the GP Iib-IIIa receptor) lack both primary and secondary responses to various platelet agonists (27).