Partial myeloperoxidase deficiency in preleukemia.
In seven subjects with partial and apparently acquired form of myeloperoxidase (MPO) deficiency, some functional properties of neutrophils (PMNs) were studied. Five patients suffered from preleukemia, one from diabetes mellitus and one from carcinoma of the breast with bone marrow metastases. Intracellular bactericidal activity, oxygen consumption and superoxide radical production were within normal limits. In three patients with preleukemia, the serum opsonic activity was markedly reduced (less than m-3SD) in an autologous system, but normal in the presence of pooled normal serum. Decreased opsonic activity was also found when these patient's sera were assayed in the presence of normal PMNs. Since the levels of IgG and C3 were comparable in the patients' sera and the pooled serum, a deficiency of another unknown opsonin or the presence of an opsonization inhibitor has to be postulated. The partial MPO defect apparently doesn't decrease the intracellular killing of Staphylococcus aureus by PMNs. The known susceptibility to bacterial infections in preleukemia may be explained by the reduction of serum opsonization conducing to a secondary decrease of the ingestion and killing of bacteria by the PMNs.
D001770:Blood Bactericidal Activity; D003165:Complement System Proteins / Q000032:analysis; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007962:Leukocytes / Q000201:enzymology* / Q000276:immunology; D009895:Opsonin Proteins / Q000096:biosynthesis; D010101:Oxygen Consumption; D009195:Peroxidase / Q000172:deficiency*; D010544:Peroxidases / Q000172:deficiency*; D010587:Phagocytosis; D011289:Preleukemia / Q000201:enzymology* / Q000276:immunology / Q000378:metabolism
D007074:Immunoglobulin G; D009895:Opsonin Proteins; D003165:Complement System Proteins; D010544:Peroxidases; D009195:Peroxidase