Defective granulocyte chemotaxis and natural killer activity in a patient with recurrent infections

A 25-year-old male with a history of recurrent infections presented with fever, severe aplastic anaemia, splenomegaly and retroperitoneal node enlargement. Lympho-histiocytic granulomas were found in spleen, liver and lymph nodes. Granulocyte studies revealed normal morphology, severely impaired random migration and complete absence of directed locomotion. Whereas phagocytosis was slightly reduced, candidacidal activity and nitroblue tetrazolium reduction were normal. Basal granulocyte cyclic GMP levels were within the normal range while a 5-fold increase of cyclic AMP levels was observed. Numerous abnormalities were also found in the patient's lymphocytes: lack of delayed hypersensitivity, reduced response to mitogens, low OKT4/OKT8 ratio, absence of natural killer (NK) activity with normal number of cells recognized by NK-specific monoclonal antibodies. These observations describe a distinct clinico-pathologic entity and suggest the possibility of a common defect in granulocytes and in NK cells.