The effect of human recombinant granulocyte-macrophage colony stimulating factor on the proliferation and differentiation of myeloid progenitors in congenital agranulocytosis marrow cells.

Congenital agranulocytosis is a rare and frequently fatal infantile disease characterized by recurrent bacterial infections, persistent absence of neutrophils in the peripheral blood and an arrest of myeloid maturation at the promyelocyte/myelocyte stage. The effect of human recombinant Granulocyte-Macrophage colony stimulating factor (GM-CSF) alone and in combination with retinoic acid, dimethylsulphoxide or actinomycin-D on the proliferation and differentiation of bone marrow cells from a child with congenital agranulocytosis was studied. Cells were treated at a concentration of 1 x 10(5) per ml in in-vitro culture with GM-CSF alone and in combination with retinoic acid, dimethylsulphoxide or actinomycin-D for 7 days at 37 degrees C in humidified incubator containing 5% CO2 in air. GM-CSF showed a profound stimulatory effect on the proliferation of myeloid progenitors from the child bone marrow and restored colony numbers in the retinoic acid-, dimethylsulphoxide- and actinomycin-D-inhibited cultures.