Granulopoiesis in Infantile Genetic Agranulocytosis In vitro Cloning of Marrow Cells in Agar Culture

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Abstract
A 3-yr-old boy and a 2-yr-old girl with infantile genetic agranulocytosis were studied by in vitro cloning of bone marrow cells in agar culture. The patients display a normal concentration of colony forming cells, and the morphological maturation is identical with that of control marrow cultured in vitro. The marrow cells of the patients show some degree of auto-stimulation indicating that endogenous production of colony stimulating factor is operating. Since an inverse relationship is expected between the peripheral neutrophil count and the percentage of marrow colony forming cells in S-phase, a high percentage was expected. The percentage of colony forming cells in S-phase was, however, extremely low, indicating a genetic unresponsiveness of granulopoietic precursor cells to feed back regulation in infantile genetic agranulocytosis.