Granulocytic progenitor cells (CFC) in a child with infantile genetic agranulocytosis (IGA) and in phenotypically normal parents.

Granulocytic progenitor cells (CFC) from the bone marrow of a child with infantile genetic agranulocytosis (IGA) were cultured twice in vitro, and the bone marrow colony-forming ability was found to be elevated. Nevertheless, when the colony-forming ability was considered in view of the differential marrow cell count and the level of circulating granulocytes, an ineffective granulopoiesis was suggested. In this patient, the blood culture showed a high number of circulating CFC. Bone marrow and blood CFC cultures were also done for the child's two parents, who were phenotypically normal and unquestionably blood-related. The number of circulating CFC was low for the father and normal for the mother. The marrow colony-forming ability of both parents was greatly reduced at the time of two cultures. This fact could constitute a way to detect the risk of IGA in descendants of blood-related subjects.