STUDIES IN SICKLE CELL ANEMIA

[摘要] Volumes of plasma and blood cells were determined in 16 patients with sickle cell anemia during crises and severe anemic episodes. Our preliminary observations permit the following conclusions:The total volume of blood in patients with sickle cell anemia was found to be slightly greater than that in the normal children used as controls. In patients with sickle cell anemia the plasma volume increased to compensate for the lowered cellular volume. A consistent increase in blood volume was observed in determinations made 24 hours after transfusions of whole blood. After transfusions of whole blood there was an increase in the total circulating mass of hemoglobin which could not be accounted for entirely by the estimated hemoglobin content of the transfused blood.Clinical improvement was noted within 24 hours in 6 of 9 patients who received infusions of 6 per cent dextran in 5 per cent fructose. Improvement in symptoms did not necessarily parallel the increase in blood volume measured at 24 hours. For example, of the 6 patients who showed clinical improvement, only 3 had significant increases in blood volume after 24 hours.It is of interest that a solution of 6 per cent dextran in 5 per cent fructose caused an increase in the total mass of hemoglobin in 6 of 9 cases studied. The volume-expanding effect of transfusions of whole blood upon the circulation may be important in ameliorating the clinical symptoms. It is possible that administration of whole blood and of solutions of 6 per cent dextran in 5 per cent fructose have a similar effect in mobilizing erythrocytes which have become trapped or stagnated in organ depots and/or small peripheral vessels.In view of the abnormally high mass of total plasma proteins (despite normal concentration per unit volume), it is postulated that infusions of dextran may exert a "protein sparing" effect in relationship to liver function.Clinically, there were no toxic, untoward or allergic reactions observed despite a tendency to prolongation of the clotting time following infusions of dextran.

[学科分类] 儿科学