Coagulation defects and platelets function in splenectomized and non-splenectomized beta-thalassemic children

ABSTRACT

The aim of this work is to evaluate the coagulation defects and platelet function in beta-thalassemic children before and after splenectomy. Also to evaluate the effect of L-carnitine therapy on the coagulation events and platelet function in these patients. The study included 56 beta-thalassemic children who were admitted at the Hematology Unit in the Pediatric University Hospital - Assiut with 20 sex and age matched children as controls. They were subjected to clinical examination and laboratory investigations in the form of screening tests for coagulation PT, APTT TT, PC and fibrinogen, natural anticoagulants studies like protein S, protein C and antithrombin III activity and platelet response to adinosindiphosphate [ADP], ristocetin [Rist], collagen and arachidonic acid [AA]. Nineteen children did splenectomy and these studies were done after 2 months of this manover. L-Carnitine therapy in a dose of 3 gm orally daily for 2 months was given to 20 non-splenectomized beta-thalassernic children and the previous follow up laboratorial studies were done. It was found that platelet response to ADP, Rist, collagen and AA was significantly decreased in non-splenoctomized patients and significantly increased in the splenectomized ones in comparison to controls. The coagulation screen tests showed significant defect in the form of prolonged PT, APTT, TT and low PC with decrease fibrinogen levels in both splenectomized and nonsplenecloinized beta-thalassemic children in comparison to controls with no significant difference between before and after splenectomy. Natural anticoagulants except protein S were significantly lower in both non-splenectomized and splenectomized beta-thalassemic children than those of the controls with no difference between before and after splenectomy. L-Carnitine therapy improved the platelet aggregation in the non splenectomized beta-thalasseinic children with no affection on the coagulation tests and natural anticoagulants. In conclusion, thalassemia major can be complicated by defective platelet aggregation and bleeding tendency. Splenectomy causes increased platelet aggregation which with the decreased levels of natural anticoagulants lead to the liability to thromboembolic complications. L-Carnitine might affect and improve platelet aggregation in non-splenectomized children but it could aggravate the thromboembolic complications in the non-splenectomized ones. Iron overload prevention with iron celation and antioxidants is recommended to prevent platelet aggregation and coagulation defects. Salicylates therapy to cases with thromboembolic complications might decrease thrombus formation and dangerous events