Erythrocyte sedimentation rate during steady state and painful crisis in sickle cell anemia

To define its diagnostic utility in sickle crisis, the erythrocyte sedimentation rates of oxygenated blood were studied in patients with sickle cell anemia and healthy normal subjects using the Guest-Westergren method. A normal range for sedimentation rate as a function of hematocrit was established in 22 normal subjects. Twenty-seven asymptomatic patients with sickle cell anemia had abnormally low sedimentation rates in relation to their hematocrits. Those low sedimentation rates were not increased by substituting plasma from healthy control subjects, which suggests that the low sedimentation rate was a cell-related phenomenon. Sedimentation rates measured in 28 patients with sickle cell anemia at the end of uncomplicated painful crisis increased to levels appropriate for their degree of anemia. In patients with sickle crisis and medical complications, the sedimentation rates were even higher. At the end of an uncomplicated painful crisis, the mean plasma fibrinogen level was significantly higher than at the onset (p less than 0.005). When red cells from patients with sickle cell anemia at the end of crisis were suspended in normal plasma from control subjects, the sedimentation rates remained high. It is concluded that the erythrocyte sedimentation rate of asymptomatic patients with sickle cell anemia is abnormally low due to cellular factors, and the increase during painful crisis is due primarily to red cell changes, modified by plasma factors.