Thrombosis has frequently been described in pathologic specimens from patients with sickle cell disease, but the contribution of this process to disease pathophysiology during life has been difficult to assess. Alterations of blood coagulation, fibrinolysis, and platelet function in sickle cell disease have now been described. Although data have often been conflicting or inconclusive, there is evidence to support the thesis that the abnormalities described are secondary phenomena that derive from small-vessel occlusion by sickled cells and resultant widespread endothelial damage. Recent documentation of increased platelet consumption and turnover during crisis suggests a possible therapeutic role for drugs that inhibit platelet function.
Rickles FR, O'Leary DS. Role of Coagulation System in Pathophysiology of Sickle Cell Disease. Arch Intern Med. 1974;133(4):635-641. doi:10.1001/archinte.1974.00320160129011