title: Retinoic acid induces granulocytic differentiation of myeloid progenitors in congenital agranulocytosis bone marrow cells.
abstract: Congenital agranulocytosis is a rare fatal infantile disease characterised by recurrent bacterial infections, persistent 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 1 microM 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 1 microM. These results suggest a potential therapeutic role for retinoic acid in the treatment of congenital agranulocytosis.
authors.affiliation: Department of Haematology, University of Cambridge Clinical School England, U.K.
authors.forename: H T
authors.initials: HT
authors.lastname: Hassan
authors.forename: E C
authors.initials: EC
authors.lastname: Pearson
authors.forename: J K
authors.initials: JK
authors.lastname: Rees
mesh_terms: D000380:Agranulocytosis / Q000151:congenital* / Q000473:pathology; D001853:Bone Marrow; D002454:Cell Differentiation / Q000187:drug effects; D002478:Cells, Cultured; D004305:Dose-Response Relationship, Drug; D005260:Female; D006098:Granulocytes / Q000473:pathology*; D006412:Hematopoietic Stem Cells / Q000473:pathology*; D006801:Humans; D007223:Infant; D014212:Tretinoin / Q000494:pharmacology*
chemical_list: D014212:Tretinoin