Platelet haemostatic properties in ß-thalassaemia: the effect of blood transfusion.

ABSTRACT

BACKGROUND: Patients with thalassaemia may have thromboembolic events and, even without thrombosis, they have a subclinical hypercoagulable state. In this setting, several coagulation laboratory abnormalities have been described, but thus far no studies have explored the contribution of platelet adhesive and procoagulant properties to blood clotting activation. In this study, we dissected the platelet procoagulant effect and influence of blood transfusions on haemostasis and platelet function in thalassaemic patients. MATERIAL AND METHODS: Sixteen subjects with thalassaemia were studied (9 with transfusion-dependent ß-thalassaemia, 7 "trait" carriers). Splenectomised and non-splenectomised patients undergoing blood transfusion were compared. All splenectomised patients were then compared to "trait" carriers and to healthy controls (n=9). The following parameters were measured in transfusion-dependent patients before and after monthly transfusions and compared to those of controls levels of platelet surface activation markers (P-selectin, tissue factor, and fibrinogen), whole blood platelet aggregation, tissue factor or adenosine diphosphate (ADP)-induced platelet thrombin generation (TG) potential, and D-dimer. RESULTS: Before transfusion, platelets from splenectomised patients showed significantly higher ADP-induced tissue factor expression, ADP- and collagen-induced platelet aggregation and TG potential than those from non-splenectomised patients and controls. Blood transfusion in splenectomised patients reduced platelet activation, aggregation and TG potential. DISCUSSION: Splenectomised patients with ß-thalassaemia had a prothrombotic state, characterised by enhanced platelet reactivity and function, and high platelet-induced TG potential. One hour after blood transfusions platelet and coagulation parameters improved, supporting the hypothesis that transfusion might have a protective role on platelet haemostatic status.