The Determinants of Irreversibly Sickled Cells in Homozygous Sickle Cell Disease

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Abstract
The relationship between the irreversibly sickled [human] cell (ISC) count and other hematological parameters was investigated. Positive correlations occurred with the MCH [mean cell Hb], MCHC [mean cell Hb concentration] and with 2 expressions of intracellular HbS 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 Hb and HbF. The MCHC affected the relationship between HbF and ISC count, a low MCHC being associated with, and probably determining, lower ISC counts at any level of HbF. A low MCHC may have inhibited ISC formation and the practical implications of this were discussed.