The determinants of irreversible sickled cells in homozygous sickle cell disease

ABSTRACT

The relationship between the irreversible sickled cell (ISC) count and other haematological parameters has been investigated. Positive correlations occurred with MCH, MCHC, and with two expressions of intracellular Hb S content. Since the ISC has a high MCHC, the positive correlations with MCHC and with factors derived from the MCHC may be difficult to evaluate. Negative correlations occurred with total haemoglobin and Hb F. The MCHC was found to affect the relationship between HbF and ISC count, a low MCHC being associated with, and probably determining, lower ISC counts at any level of Hb F. It is proposed that a low MCHC may inhibit ISC formation and the practical implications of this are discussed (AU)