We examined platelet aggregation in platelet-rich plasma (PRP) and in whole blood from two patients with Glanzmann's thrombasthenia. In PRP, aggregation was measured by monitoring the changes in light absorbance that occurred in response to aggregating agents; to measure platelet aggregation in whole blood, we used a platelet counting technique. In PRP, the patients' platelets showed defective ...

Two Hispanic siblings presenting with lifelong mucocutaneous bleeding were diagnosed clinically with Glanzmann thrombasthenia on the basis of a normal platelet count, prolonged bleeding time and absent platelet aggregation in response to multiple agonists. Quantitative analysis of the probands' platelets by flow cytometry showed a complete absence of GPIIb-IIIa, consistent with Type I thrombast...

The platelets of 11 patients with Glanzmann's thrombasthenia and their nearest family members were studied for the expression of the platelet-specific alloantigens of the Zw-, Ko- and Bak systems. The strength of the expression of the Zwa antigen was diminished on the platelets of 3 patients, and the antigen was absent from the platelets of the other 8. The platelets of none of the patients rea...

In an epileptic patient with malacia from intracerebral hemorrhage in left parietal and occipital lobes due to thrombasthenia, TP occurred following treatment of antiepileptic drug CBZ. Based on the dynamic changes of clinical manifestation and dosage of CBZ, in combination with the test results of D-Dimer, fibrinogen, prothrombin degradation products, and other laboratory tests, especially of ...

We studied the defect responsible for Glanzmann thrombasthenia in a patient whose platelets expressed < 5% of the normal amount of GPIIb-IIIa. Genetic and biochemical evidence indicated that the patient's GPIIIa genes were normal. However, DNA analysis revealed the patient homozygous for a G818-->A substitution in her GPIIb genes, resulting in a Gly273-->Asp substitution adjacent to the first G...

A 41-year-old man with an unremarkable medical history presented with a painful knee after a sports injury. He was diagnosed with a medial meniscal tear. Symptoms did not abate after 6 months of physical therapy, and he underwent arthroscopic partial medial meniscectomy. A week after beginning physical therapy he experienced a knee effusion, decreased ROM, and inability to flex his quadriceps. ...

Patient C.M. presented platelet function defects symptomatic of Glanzmann's thrombasthenia. However, analysis of surface-labeled platelets by SDS-polyacrylamide gel electrophoresis revealed the usual presence of the major glycoproteins, including GP IIb and GP IIIa. Platelet fibrinogen was not detected. Analysis of Triton X-100 extracts of Ca2+-washed C.M. platelets by crossed immunoelectrophor...