Treatment of adult systemic mastocytosis with interferon-α: results of a multicentre phase II trial on 20 patients

Autor: Philippe Casassus, Loïc Guillevin, Martine Raphael, Patrice Beaudry, Antoine Martin, Pierre Lortholary, Liliane Laroche, Nadine Caillat-Vigneron, Virginie Eclache, Valérie Gallais, Jeanne Simon, Olivier Lortholary
Rok vydání: 2002
Předmět:
Zdroj: British Journal of Haematology. 119:1090-1097
ISSN: 0007-1048
DOI: 10.1046/j.1365-2141.2002.03944.x
Popis: Systemic mastocytosis (SM) is characterized by proliferation of mast cells in various organs, which may release a wide variety of mediators, thereby explaining the broad clinical spectrum of disease manifestations. The potentially life-threatening systemic symptoms and tumoral proliferation are poorly controlled despite the use of several cytotoxic chemotherapies and/or symptomatic treatments. Twenty consecutive adult SM patients with histologically confirmed bone marrow (BM) involvement received interferon-alpha subcutaneously (1-5 million units/m2/d, with progressive dose intensification over the first month of treatment) and were evaluated after 6 months of therapy. Seven of them had previously received symptomatic treatments, including steroids, which were ineffective. Among the 13 patients treated for at least 6 months, seven partial and six minor responses, mainly concerning vascular congestion and skin lesions, were obtained, while BM infiltration remained unchanged in 12 patients. The significant reduction of mast-cell mediator levels after 6 months of treatment was not predictive of clinical remission. The rate of depression was unexpectedly high (seven patients; 35%). Two patients died soon after starting therapy (one myocardial infarction, one septic shock). Six months of interferon-alpha may relieve vascular congestion in adults with SM, probably by inhibiting mast-cell degranulation.
Databáze: OpenAIRE
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