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Function of Granulocytes with Deficient Myeloperoxidase‐Mediated Iodination in a Patient with Generalized Pustular Psoriasis
Author(s) -
Stendahl Olle,
Lindgren Sören
Publication year - 1976
Publication title -
scandinavian journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0036-553X
DOI - 10.1111/j.1600-0609.1976.tb01130.x
Subject(s) - myeloperoxidase , granulocyte , candida albicans , intracellular , chronic granulomatous disease , chemistry , biochemistry , peroxidase , enzyme , microbiology and biotechnology , medicine , immunology , endocrinology , biology , inflammation
The granulocytes of a patient with generalized pustular psoriasis (GPP) were found to have impaired ability to fix iodine after ingestion of yeast particles. Since hexose monophosphate shunt (HMS) activity was increased and the contents of 3 other lysmomal enzymes, β‐glucuronidase, N‐acetyl‐β‐glucosaminidase and lysoeyme were within normal range, the impaired iodination appeared to be due to a selective defect of myeloperoxidase (MPO) activity within the phagocytic cells. The deficient iodination was accompanied by a decreased intracellular killing of E. coli and C. albicans. Since hexose monophosphate shunt activity was enhanced and azide and cyanide inhibited the intracellular killing of E. coli only moderately, the patient's granulocytes may possess azide‐ and cyanide‐resistant, MPO‐independant microbicidal systems coupled to the oxidative metabolism. Assessment of granulocyte iodination and enzyme contents of the relatives of the patient revealed no hereditary transmission. Since GPF is characterized by the development of subcorneal pustules containing granulocytes, the MPO‐deficiency may be the cause of or enhance the development of the disease.