ATYPICAL NEUTROPHIL ALKALINE-PHOSPHATASE ASSOCIATED WITH IMPAIRED NEUTROPHIL FUNCTIONS

We report the case of a healthy young man presenting with atypical neu trophil alkaline phosphatase (NAP) and reduced neutrophil chemotactic activity, but with no susceptibility to infection. NAP activity was lo w, kinetic parameters were modified and immunoreactive properties and subcellular distribution were abnormal. Neutrophil morphology was norm al. A similar pattern was observed in the patient's healthy brother. T he profile of the observed anomalies offers some similarity to that pr eviously described in patients with chronic myelogenous leukaemia. How ever, in the present case, the NAP deficiency with impaired neutrophil function was present in two brothers with no haematological symptoms and is probably related to a non-acquired neutrophil abnormality. This observation of a primary NAP variant reinforces the hypothesis of a d irect link between NAP activity and functional properties of neutrophi ls. (C) 1998 Elsevier Science B.V. All rights reserved.