Chronic Granulocytopenia in Childhood

Observations in five cases of chronic benign granulocytopenia are reported. The disorder appears to be a true entity occurring in early life. Despite severe depression of the circulating granulocyte count, the course is benign because a reserve of immature granulocytes is available in the bone marrow. Depletion of the leukocyte storage pool by increased peripheral destruction, possibly enhanced by sequestration, rather than a maturation arrest at the penultimate stage is the probable mechanism. This process appears to be of moderate intensity, permitting the maintenance of a "steady state" of low efficiency, resulting in increased susceptibility but not total lack of resistance to infection. The disorder represents a natural model of a special disturbance in leukokinetics. Its ultimate cause remains unknown but is probably related to age-determined factors.