Granulocyte-macrophage Colony Stimulating Factor Does Not Improve Neutrophil Oxidative Metabolism in a Patient with Variant X-linked Chronic Granulomatous Disease

Overview

Journal Eur J Pediatr

Specialty Pediatrics

Date 1991 Jun 1

PMID 1659535

Citations 4

Authors

T J Muhlebach

H J Feickert

K Welte

R A Seger

Affiliations

Soon will be listed here.

Abstract

Variant X-linked chronic granulomatous disease (CGD) is characterised by a decreased but still measurable respiratory burst and cytochrome b content of phagocytes resulting in a clinically milder form of the disease. We examined the in vivo effect of recombinant human granulocyte-macrophage colony stimulating factor (rh-GM-CSF) on the neutrophil functions of a patient treated for liver abscess. The number of white blood cells was markedly increased at the highest dose of GM-CSF injected (30 micrograms/kg per day). This was mainly due to a large increase in eosinophils and to a lesser extent in neutrophils. No change in the deficient neutrophil respiratory burst nitroblue tetrazolium (NBT)-reduction, superoxide (O2-)-production and cytochrome b content was observed during 6 weeks of therapy with increasing doses of GM-CSF. No significant clinical improvement of the liver abscess was observed during treatment with GM-CSF.

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References

Newburger P, Ezekowitz R . Cellular and molecular effects of recombinant interferon gamma in chronic granulomatous disease. Hematol Oncol Clin North Am. 1988; 2(2):267-76. View

Weisbart R, Golde D, Clark S, Wong G, Gasson J . Human granulocyte-macrophage colony-stimulating factor is a neutrophil activator. Nature. 1985; 314(6009):361-3. DOI: 10.1038/314361a0. View

Ezekowitz R, Newburger P . New perspectives in chronic granulomatous disease. J Clin Immunol. 1988; 8(6):419-25. DOI: 10.1007/BF00916946. View

Ezekowitz R, Orkin S, Newburger P . Recombinant interferon gamma augments phagocyte superoxide production and X-chronic granulomatous disease gene expression in X-linked variant chronic granulomatous disease. J Clin Invest. 1987; 80(4):1009-16. PMC: 442339. DOI: 10.1172/JCI113153. View

Weisbart R, Kwan L, Golde D, Gasson J . Human GM-CSF primes neutrophils for enhanced oxidative metabolism in response to the major physiological chemoattractants. Blood. 1987; 69(1):18-21. View

HITZIG W, Seger R . Chronic granulomatous disease, a heterogeneous syndrome. Hum Genet. 1983; 64(3):207-15. DOI: 10.1007/BF00279395. View

Kitagawa S, Yuo A, Souza L, Saito M, Miura Y, Takaku F . Recombinant human granulocyte colony-stimulating factor enhances superoxide release in human granulocytes stimulated by the chemotactic peptide. Biochem Biophys Res Commun. 1987; 144(3):1143-6. DOI: 10.1016/0006-291x(87)91430-6. View

Lutter R, van Schaik M, van Zwieten R, Wever R, Roos D, Hamers M . Purification and partial characterization of the b-type cytochrome from human polymorphonuclear leukocytes. J Biol Chem. 1985; 260(4):2237-44. View

Parkos C, Dinauer M, Jesaitis A, Orkin S, Curnutte J . Absence of both the 91kD and 22kD subunits of human neutrophil cytochrome b in two genetic forms of chronic granulomatous disease. Blood. 1989; 73(6):1416-20. View

10.

Klausmann M, Pfluger K, Krumwieh D, Seiler F, Havemann K . Stimulation of oxidative metabolism of granulocytes by recombinant granulocyte-macrophage-colony-stimulating-factor and a conditioned medium of a urinary bladder carcinoma cell line. Blut. 1987; 54(5):307-12. DOI: 10.1007/BF00320879. View

11.

Sechler J, Malech H, White C, Gallin J . Recombinant human interferon-gamma reconstitutes defective phagocyte function in patients with chronic granulomatous disease of childhood. Proc Natl Acad Sci U S A. 1988; 85(13):4874-8. PMC: 280539. DOI: 10.1073/pnas.85.13.4874. View

12.

Klausmann M, Pfluger K, Krumwieh D, Seiler F, Havemann K . Influence of recombinant human granulocyte-macrophage colony-stimulating factor on granulocyte functions. Behring Inst Mitt. 1988; (83):265-9. View

13.

Roos D, de Boer M . Purification and cryopreservation of phagocytes from human blood. Methods Enzymol. 1986; 132:225-43. DOI: 10.1016/s0076-6879(86)32010-x. View

14.

Ezekowitz R, Dinauer M, Jaffe H, Orkin S, Newburger P . Partial correction of the phagocyte defect in patients with X-linked chronic granulomatous disease by subcutaneous interferon gamma. N Engl J Med. 1988; 319(3):146-51. DOI: 10.1056/NEJM198807213190305. View

15.

Curnutte J, Babior B . Chronic granulomatous disease. Adv Hum Genet. 1987; 16:229-97. DOI: 10.1007/978-1-4757-0620-8_4. View

16.

English D, Broxmeyer H, Gabig T, Akard L, Williams D, Hoffman R . Temporal adaptation of neutrophil oxidative responsiveness to n-formyl-methionyl-leucyl-phenylalanine. Acceleration by granulocyte-macrophage colony stimulating factor. J Immunol. 1988; 141(7):2400-6. View

17.

Metcalf D . The molecular biology and functions of the granulocyte-macrophage colony-stimulating factors. Blood. 1986; 67(2):257-67. View

18.

Clark S, Kamen R . The human hematopoietic colony-stimulating factors. Science. 1987; 236(4806):1229-37. DOI: 10.1126/science.3296190. View

19.

Bohler M, Seger R, Mouy R, Vilmer E, Fischer A, Griscelli C . A study of 25 patients with chronic granulomatous disease: a new classification by correlating respiratory burst, cytochrome b, and flavoprotein. J Clin Immunol. 1986; 6(2):136-45. DOI: 10.1007/BF00918746. View