Hereditary myeloperoxidase deficiency
P Cech, A Papathanassiou, G Boreux, P Roth and PA Miescher
The functional properties of granulocytes in a diabetic patient deficient
in myeloperoxidase (MPO) were compared with those of granulocytes in
healthy subjects. The granulocytes of this patient had normal phagocytic
activity. The microbicidal activity of the granulocytes was partially
diminished with regard to Staphylococcus aureus and was almost nil with
regard to Candida albicans. Fungicidal activity of normal granulocytes was
shown to be impaired during the in vitro artificial hyperglycemic
condition. The relationship among diabetes mellitus, MPO deficiency, and
serious C. albicans infection was examined. Genetic investigation was
carried out in 28 members of the proband's family. In close relatives of
the patient, MPO values were found to be diminshed to a greater or lesser
degree, thus suggesting variable expressivity of the heterozygote state of
MPO deficiency.
Volume 53,
Issue 3,
pp. 403-411,
03/01/1979
Copyright © 1979 by The American Society of Hematology
