Summary
Thromboembolic complications are not uncommon in thalassemia. Previous studies suggest
increased platelet aggregation and a potential role of pathological changes in the
red blood cell (RBC) lipid membrane, induced by oxidative stress. In the present study,platelet
adhesion and the effect of thalassemic RBC on platelet adhesion under flow conditions
were evaluated, using the Cone and Plate (let)Analyzer(CPA).Twenty-two β-thalasse-mia
patients and 22 blood type-matched healthy controls were studied. An increased platelet
adhesion (% surface coverage, SC),was observed in patients as compared to controls
(p<0.05). When platelet count and haematocrit were normalized by auto-logous reconstitution,
a significant increase in platelet aggregation (average size,AS) was observed (p<0.05).
Increased platelet adhesion (SC and AS), was demonstrated in six patients with a history
of thrombosis as compared to 16 patients without any history of thrombosis (p≤0.007)
and in 17 splenectomized patients as compared to five non-splenectomized patients
(p=0.003). In reconstitution studies, thalassemic RBC mixed with normal platelet-rich
plasma significantly increased platelet adhesion compared to normal RBC (SC p<0.03,
AS p<0.02). Thalassemic platelets reconstituted with normal RBC, had increased aggregation
(AS, p<0.004) in comparison with normal platelets.The results indicate that increased
platelet adhesion in β-thalassemia is induced by both platelets and RBC. Increased
platelet adhesion correlated with clinical thrombotic events and thus may suggest
a mechanism of thrombosis in thalassemic patients.The potential application of the
CPA in identifying thalassemic patients with high risk for thrombosis should be studied
prospectively in a larger cohort of patients.
Keywords
β-thalassemia - thrombosis - red blood cells - platelets - adhesion - aggregation