Evaluation of Platelet Surface Glycoproteins in Patients With Inherited Thrombocytopathy: Association With Aggregation Studies and Bleeding Severity

Disorders of platelet function are characterized by variable mucocutaneous bleeding manifestations and excessive hemorrhage following surgical procedures or trauma. Generally, most patients have mild to moderate bleeding manifestations with a prolonged bleeding time.

Platelet aggregation and secretion studies using platelet-rich plasma (PRP) provide evidence for platelet dysfunction but are neither predictive of severity of clinical manifestations nor the molecular mechanisms.

Glanzmann's thrombasthenia (GT) is a rare autosomal recessive genetic bleeding syndrome characterized by defects in platelet aggregometry. The clinical phenotype of patients with GT is variable. Some suffer from severe bleeding, while others have only mild bleeding. Some studies found bleeding severity in GT was influenced by the abundance and functioning of platelet receptors involved in aggregation and adhesion.

In addition to a complete medical history, a GT diagnosis requires a comprehensive laboratory workup, including platelet aggregation analysis, and a confirmation by flowcytometry or western blotting with monoclonal antibodies that recognize the GPIIb/IIIa complex.

Platelet flow cytometry is an emerging tool in diagnostic and therapeutic hematology. It is eminently suited to study the expression of platelet surface receptors both qualitatively as well as quantitatively.

Aim of the study:-

• Determine the role of flowcytometry as a quantitative measurement tool of platelets surface glycoproteins in patients with inherited thrombocytopathies and its correlation with bleeding severity of these patients.
• To compare the efficacy, advantages and disadvantages between platelets flowcytometry and aggregometer in diagnosing various inherited thrombocytopathies.