title: Interest of interferon alpha in systemic mastocytosis. The French experience and review of the literature.
abstract: Systemic mastocytosis (SM) are defined by an abnormal growth and accumulation of mast cells in bone marrow and/or other extracutaneous organs. There is currently no cure for this disease. Because of similarities and/or association of mastocytosis with myeloproliferative disorders, interferon alpha has been tested but with contradictory reported results. A first prospective multicenter phase II trial was then started in France. From 1994 to 1997, 20 adult patients with confirmed bone marrow involvement received interferon alpha-2b for at least 6 months, (from 1 million U per day up to 5 million U/m(2)/day). Thirteen patients who presented systemic and/or specific cutaneous manifestations, demonstrated objective responses: seven (35%) were partial, six (30%) minor but no complete response could be observed at the time of analysis. The bone marrow remained unchanged in 12/13. Thus, interferon should be offered to patients with severe systemic manifestations, who have not responded to symptomatic therapies, even in case of non-aggressive mastocytosis, with or without corticosteroids the first weeks. Long-term therapy should be offered to patients with initial positive response. To control more aggressive SM or mastocytosis associated with clonal hematologic non-mast cell lineage or leukaemia mast cell, other chemotherapeutic regimens should be proposed like Cladribine (2-chlorodeoxyadenosine, 2-CDA) or polychemotherapies including interferon as it is being tested in France in a new multicentric protocol, coordinated by the association AFIRMM, with interferon and oral cytarabine.
authors.affiliation: Service de médecine Interne-néphrologie, Centre hospitalier Intercommunal de Poissy-Saint-Germain-en-Laye, 10, rue Champ Gaillard, 78300 Poissy, France. jsimon@chi-poissy-st-germain.fr
authors.forename: J
authors.initials: J
authors.lastname: Simon
authors.forename: O
authors.initials: O
authors.lastname: Lortholary
authors.forename: N
authors.initials: N
authors.lastname: Caillat-Vigneron
authors.forename: M
authors.initials: M
authors.lastname: Raphaël
authors.forename: A
authors.initials: A
authors.lastname: Martin
authors.forename: J
authors.initials: J
authors.lastname: Brière
authors.forename: S
authors.initials: S
authors.lastname: Barète
authors.forename: O
authors.initials: O
authors.lastname: Hermine
authors.forename: Ph
authors.initials: P
authors.lastname: Casassus
mesh_terms: D001854:Bone Marrow Cells / Q000473:pathology; D005602:France; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use*; D008407:Mast Cells / Q000473:pathology; D034721:Mastocytosis, Systemic / Q000188:drug therapy* / Q000473:pathology
chemical_list: D016898:Interferon-alpha