Coagulation factor XIII concentration in sickle-cell disease.

Plasma factor XIII was assayed serially for a 1 1/2 to 2 year period in four patients with sickle-cell disease (SSD), during which several episodes of SSD crisis were observed. During crisis-free periods, plasma factor XIII concentration was lower than in normal subjects. With crisis onset, there was usually a concomitant fall in platelets and in plasma factor XIII soon after followed by a rise in fibrinogen concentration and platelet count. Following crisis resolution there was an increase in plasma factor XIII concentration peaking 3 weeks after crisis onset and occurring at the time of subsidence of thrombocytosis. It is suggested that the observed changes in plasma factor XIII concentration in SSD patients support the hypothesis that in the early stage of SSD crisis, fibrin deposition and/or thrombosis significantly enhance the role of sickled erythrocytes in producing vascular occlusion and/or organ infarction.