title: Treatment of systemic mastocytosis with interferon-gamma: failure after appearance of anti-IFN-gamma antibodies.
abstract: We report a case of a patient with systemic mastocytosis who was treated with interferon-gamma. Because of severe diarrhoea, nausea and weight loss due to mast cell infiltration of the gastric mucosa the patient received 150 micrograms d-1 interferon-gamma subcutaneously for 10 months. During therapy, the plasma concentrations of IL-3, IL-4 and GM-CSF, which seem to play a role in mast cell growth and differentiation were monitored. The patient had good symptomatic relief and the initially very high eosinophil counts in the peripheral blood showed a partial reduction. However, after 4 months of therapy the patient relapsed. In serum obtained after the relapse, but not in stored serum from the beginning of the therapy, neutralizing antibodies against interferon-gamma were found. Therefore an initial response to the therapy and a secondary failure mediated by treatment-induced antibodies against recombinant interferon-gamma might be suggested. Interferon-gamma may be a well tolerated therapeutic option in systemic mastocytosis. However, treatment-induced neutralizing antibodies against recombinant interferon-gamma should be considered if secondary treatment failure occurs.
authors.affiliation: Medizinische Klinik und Poliklinik V, Universität Heidelberg, Germany.
authors.forename: C
authors.initials: C
authors.lastname: Fiehn
authors.forename: O
authors.initials: O
authors.lastname: Prümmer
authors.forename: H
authors.initials: H
authors.lastname: Gallati
authors.forename: B
authors.initials: B
authors.lastname: Heilig
authors.forename: W
authors.initials: W
authors.lastname: Hunstein
mesh_terms: D000368:Aged; D000906:Antibodies / Q000097:blood*; D005260:Female; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000097:blood; D006801:Humans; D007371:Interferon-gamma / Q000276:immunology* / Q000627:therapeutic use*; D007377:Interleukin-3 / Q000097:blood; D015847:Interleukin-4 / Q000097:blood; D008415:Mastocytosis / Q000628:therapy*; D017211:Treatment Failure
chemical_list: D000906:Antibodies; D007377:Interleukin-3; D015847:Interleukin-4; D007371:Interferon-gamma; D016178:Granulocyte-Macrophage Colony-Stimulating Factor