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April 1974

Role of Coagulation System in Pathophysiology of Sickle Cell Disease

Author Affiliations

Washington, DC

From the Department of Hematology, Walter Reed Army Institute of Research, Walter Reed Army Medical Center (Dr. Rickles), and the Division of Hematology, Department of Medicine, and Division of Laboratory Medicine, Department of Pathology, George Washington University Medical Center, Washington, DC (Dr. O'Leary).

Arch Intern Med. 1974;133(4):635-641. doi:10.1001/archinte.1974.00320160129011

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.