[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.
D000380:Agranulocytosis / Q000150:complications / Q000151:congenital*; D003241:Consanguinity; D005260:Female; D006801:Humans; D007223:Infant; D008218:Lymphocytosis / Q000150:complications; D008231:Lymphopenia / Q000150:complications*; D006377:T-Lymphocytes, Helper-Inducer; D050378:T-Lymphocytes, Regulatory
Helfer-T-Zellmangel bei Infantiler Genetischer Agranulozytose (M. Kostmann).