Erythrocyte Calcium Abnormalities and the Clinical Severity of Sickling Disorders

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Abstract
Erythrocyte Ca levels and uptake were studied in patients with sickle hemoglobinopathies of different clinical severity to relate these measurements to the production of irreversibly sickled cells and disease severity. Erythrocyte Ca levels were measured by atomic absorption spectroscopy and Ca by isotopic means. In sickle cell anemia, erythrocyte Ca content was elevated and the uptake of isotopic Ca increased under oxygenated and deoxygenated conditions. There was a direct correlation between the numbers of irreversibly sickled cells and Ca uptake and an inverse relationship between Ca uptake and red cell K level. The clinical course of disease was milder in patients with high fetal Hb levels, but there was no relationship between clinical course and Ca levels, Ca flux or irreversibly sickled cells. Ca accumulation and irreversibly sickled cell formation are apparently related processes. The absence of good correlation between various biochemical and clinical parameters emphasizes the complexity of factors which modify the clinical course of this disorder.