Determinants of haemoglobin level in sickle cell-haemoglobin C disease

The determinants of steady state haemoglobin levels in sickle cell-haemoglobin C (SC) disease were investigated by measuring routine haematological and biochemical indices, red cell survival, oxygen affinity, pitted erythrocytes, and red cell and plasma volumes in 31 adult patients (15 male; 16 female). Red cell survival was shortened in all subjects, and was positively correlated with haemoglobin level. However, many haemoglobin values were within the normal range, especially in male subjects. Palpable splenomegaly, which occurred in 53 percent of patients, did not appear to effect haemoglobin levels, red cell survival, plasma volume, but was associated with lower platelet counts and decreased pitted red cells. Sex related differences were found in total haemoglobin, packed cell volume, conductivity cell volume, red cell count, and in the blood volume measurements. Red cell, plasma and total blood volumes in patients varied with weight and cube of height in manner observed in normal subjects, although red cell volumes were lower and plasma volumes were greater than in normal subjects of given height and weight. Anaemia in SC disease is related to the haemolytic rate but the major determinant appears to be an inappropriate increase in plasma volume (Summary)

Total body water, extracellular, and plasma volume compartments in sickle cell anaemia

The total body water (TBW), plasma (PV) and extracellular fluid volumes (ECFV) of twenty-nine subjects with sickle cell anaemia, ten of whom were in painful crisis, were studied. During asymtomatic periods (the steady state), the ECFV of subjects with the anaemia is increased when compared with normal controls, because of plasma volume expansion; the interstitial fluid volume (ISFV) is normal, and intra cellular water (ICFV) is diminished by 5 percent of weight. During painful crisis, there was a marked tendency to loss of plasma volume into the interstitial fluid compartment, and the data also suggested that there was acute cellular distruction. Most of the patients in this study managed, however, presumably by compensatory changes in renal function and fluid intake, to maintain a normal plasma volume during painful crisis (AU)

Plasma and blood volumes in severely malnourished Jamaican children

Twenty-six determinations of plasma and blood volumes were done in 7 malnourished Jamaican children, on admission to hospital and as they recovered. There was a fall in plasma volume expressed per unit of body weight but no change in blood volume. There were significant correlations between the plasma volume changes, the weight deficit, and the venous haematocrit. (AU)