Helper T-cell deficiency in infantile genetic agranulocytosis (M. Kostmann)

A female turkish infant with congenital infantile genetic agranulocytosis was examined for its distribution of lymphocyte subclasses. Imbalances of B cells and T-cell subpopulations were found. Compared to the normal control group a relative as well as an absolute deficiency of T helper cells and an increase of the absolute number of T suppressor cells were the most prominent findings. The importance of immunoregulatory T-cell subsets in the pathogenesis of infantile genetic agranulocytosis, disorders of hematopoiesis and granulocyte production, as well as primary and secondary immunodeficiencies is discussed.