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  • Title: New antisickling agents.
    Author: Labie D.
    Journal: Haematologia (Budap); 1983; 16(1-4):105-8. PubMed ID: 6679483.
    Abstract:
    Sickle cell anemia has variable clinical expression, ranging from benign cases to very severe ones. This situation suggest that, unless a perfect drug is identified, the therapeutic approaches may also vary. Great progress has been made in this last decade in the understanding of the Hb S polymer structure, the kinetics of polymerization and some of the cellular contributions to the disease. This progress has suggested that we could alter the course of the disease by impairing the polymerization of Hb S through three mechanisms: a) interfering in the areas of contacts involved in polymer formation by covalent and non-covalent agents, b) changing the oxygen affinity of Hb S to decrease the changes induced by deoxygenation, c) diluting the Hb S by increasing the MCV of the cells and consequently decreasing its MCHC. This last result is achieved by a modification of the permeability of the red cell membrane.
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