Determinants of Haemoglobin Level in Sickle Cell‐Haemoglobin C Disease

Abstract

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 occured in 53% of patients, did not appear to affect haemoglobin level, red cell survival, plasma volume, or red cell 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.

Molecular and cellular pathogenesis of hemoglobin SC disease.

“…The mean proportion ofHb S was 50% in nine SC samples analyzed by anion exchange chromatography (25). In a group of 32 samples analyzed by electrophoresis, a skewed distribution was found with a mean proportion of 58% Hb S and a range of 54-65% (26). In sickle trait individuals, there is a trimodal distribution, with a peak at 40% and progressively smaller peaks at 32% and 28% Hb S (25,27).…”