Congenital bleeding disorders with long bleeding time and normal platelet count

Glycoproteins of Platelet Membranes from Glanzmann's Thrombasthenia. A Comparison with Normal Using Carbohydrate-Specific or Protein-Specific Labelling Techniques and High-Resolution Two-Dimensional Gel Electrophoresis

“…The presence of these two glycoproteins (IIbA1 and IIIaAl) albeit in severely reduced amounts has been previously reported by the use of a number of different techniques [3,4,6,7,15]. Following Caen's nomenclature for classifying Glanzmann's thrombasthenia platelets according to the absence (type I) or reduction (type 11) in platelet clot retraction [20] it was found that Glanzmann's thrombasthenia platelets where IIbAl and IIIaAl could be detected were of type I1 and those Glanzmann's thrombasthenia platelets in which these two glycoproteins could not be detected were of type I (Revol, L. and Gross, R., unpublished observations). In our results when IIbAl and IIIaAl are present in Glanzmann's thromb- asthenia platelets they can both be detected by a proteinspecific labelling technique (Fig.…”

The molecular genetic basis of Glanzmann thrombasthenia in the Iraqi-Jewish and Arab populations in Israel.

“…Glanzmann thrombasthenia is a congenital bleeding disorder defined by defective or quantitatively abnormal platelet glycoprotein (GP) Ilb-IIIa (allb133) receptors (1)(2)(3). The diagnosis of this disorder is based on a clinical syndrome of mucocutaneous bleeding in association with abnormal platelet aggregation in response to physiologic stimuli (e.g., ADP, thrombin, collagen, and epinephrine), normal platelet counts, and prolonged bleeding times.…”

Expression of Cd41 (Gpiib) and Cd61 (Gpiiia) in Patients With Glanzmann Thrombasthenia Using Flow Cytometry

“…However, flow cytometry is another technique that has an increasing application in this field, the corresponding antibodies (anti CD41 and CD61) is directed by flow cytometry against CD41 (GPIIb) and CD61 (GPIIIa) respectively, so the percentage of these receptors expression can be determined and hence the types and sub types of G.T. can be identified (7,8) . Severe reduction (< 5% for both receptors) in the expression was consistent with type I, moderate reduction (5-20%) goes with type II whereas > 20% expression is regarded as type III (7) .…”

“…can be identified (7,8) . Severe reduction (< 5% for both receptors) in the expression was consistent with type I, moderate reduction (5-20%) goes with type II whereas > 20% expression is regarded as type III (7) . Glnazmann thrombosthenia is divided into three types: (8) Type I: severe reduction in expression of both receptors.…”