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Significance of blood coagulation and platelet profiles in relation to pulmonary thrombosis in beta-thalassemia/Hb E.

In beta-Thalassemia hemoglobin E (beta-thal Hb E), hypoxemia with abnormal lung function was described and postmortem examination in these patients showed organized pulmonary trombi with thickened arterial wall, particularly in post-splenectomized cases. Coagulation and platelet profiles were studied in 58 beta-thal Hb E patients. In 35 cases with intact spleen, the fibrinolytic activity was significantly decreased with high antithrombin III activity, while coagulation tests revealed mild abnormality. The platelet aggregation to ADP, adrenaline, collagen and ristocretin were defective and platelet 5-hydroxytryptamine content was lower than normal. Twenty-three patients who had been splenectomized for 5-18 years, decreased fibrinolytic activity and high antithrombin III activity were also observed. The coagulation profiles and platelet aggregation in response to ADP, adrenaline and collagen showed better results. Fourteen cases exhibited thrombocytosis and their thrombin generation was in the hypercoagulable range. Platelet aggregation in response to ristocetin remained defective and platelet 5-hydroxytryptamine content was lower than in cases with intact spleens. Defective aggregation to ristocetin would indicate abnormal von Willebrand's factor (vWF). Decreased fibrinolysis should very likely have a role in the occurrence of thrombosis and the better hemostatic profiles in post-splenectomized cases would contribute to the more frequent thrombotic incidence in these cases.