Atypical neutrophil alkaline phosphatase associated with impaired neutrophil functions

We report the case of a healthy young man presenting with atypical neutrophil alkaline phosphatase (NAP) and reduced neutrophil chemotactic activity, but with no susceptibility to infection. NAP activity was low, kinetic parameters were modified and immunoreactive properties and subcellular distribu...

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Bibliographic Details
Published in:Clinica chimica acta 1998-09, Vol.277 (1), p.77-89
Main Authors: Laharrague, Patrick, Denier, Colette J., Grozdea, Jean, Perié, Jacques-Jean, Corberand, Joël X., Fillola, Geneviève, Vergnes, Henri
Format: Article
Language:English
Subjects:
Adult
Alkaline Phosphatase - antagonists & inhibitors
Alkaline Phosphatase - chemistry
Alkaline Phosphatase - deficiency
Biological and medical sciences
Cell Nucleus - enzymology
Chelating Agents
Chemotaxis, Leukocyte
Cytoplasm - enzymology
Dimerization
Edetic Acid - pharmacology
Electron microscopy
Enzyme Inhibitors - pharmacology
Fundamental and applied biological sciences. Psychology
Fundamental immunology
Granulocytes
Humans
Immunobiology
Kinetic studies
Male
Microscopy, Electron
Myeloid cells: ontogeny, maturation, markers, receptors
Neuraminidase - pharmacology
Neutrophil alkaline phosphatase
Neutrophil functions
Neutrophils - enzymology
Neutrophils - physiology
Neutrophils - ultrastructure
Polynuclears
Urea - pharmacology
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Summary:We report the case of a healthy young man presenting with atypical neutrophil alkaline phosphatase (NAP) and reduced neutrophil chemotactic activity, but with no susceptibility to infection. NAP activity was low, kinetic parameters were modified and immunoreactive properties and subcellular distribution were abnormal. Neutrophil morphology was normal. A similar pattern was observed in the patient's healthy brother. The profile of the observed anomalies offers some similarity to that previously described in patients with chronic myelogenous leukaemia. However, 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 direct link between NAP activity and functional properties of neutrophils.
ISSN:0009-8981
1873-3492
DOI:10.1016/S0009-8981(98)00119-3