Coagulation profile was suggestive of Glanzmann thrombasthenia
and pregnancy-report of a case and literature review.
Excessive gingival bleeding in two patients with 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
Use of rFVIIa in 4 children with Glanzmann thrombasthenia
For example, Glanzmann thrombasthenia
is a deficiency of the IIb and/or IIIa subunits of the GP IIb/IIIa 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).
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.
, 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).