Abstract
Congenital agranulocytosis is a rare fatal infantile disease characterised by recurrent bacterial infections, persistant absence of neutrophils and maturation arrest at the promyelocyte/myelocyte stage. The effectiveness of retinoic acid in inducing differentiation of congenital agranulocytosis marrow myeloid progenitor cells was studied. Non-adherent mononuclear marrow cells were treated in an in vitro culture with retinoic acid at various concentrations from 1nM to 1μM for seven days. Morphological and functional differentiation into mature granulocytes was induced by retinoic acid in a dose-response stimulation with a maximum response at a concentration of 1μM. These results suggest a potential therapeutic role for retinoic acid in the treatment of congenital agranulocytosis.
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