thrombasthenia

(redirected from Glanzmann thrombasthenia)
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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 ?
Coagulation profile was suggestive of Glanzmann thrombasthenia.
Glanzmann thrombasthenia and pregnancy-report of a case and literature review.
Excessive gingival bleeding in two patients with Glanzmann thrombasthenia.
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.
FVII defciency, * FVII leve was 20%, ND: Not done, VWD: Von Willebrand disease, VWF: Von Wolebrand factor, BSS: Bernard-Soulier syndrome, GT: Glanzmann thrombasthenia
For example, Glanzmann thrombasthenia is a deficiency of the IIb and/or IIIa subunits of the GP IIb/IIIa receptor.
Intensive and Successful Transfusion of Pathogen Inactivated INTERCEPT Platelet Concentrates for Major Gynecological and Obstetrical Surgery in Glanzmann Thrombasthenia Type 1 with Gypsy Mutation documented the ability of INTERCEPT-treated platelets to support two patients with a recessive autosomal severe bleeding disorder during surgical procedures.
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.
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.
describe the defect in each of the following hereditary disorders: Glanzmann thrombasthenia and Bernard-Soulier syndrome (BSS).
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).