title: Efficacy and safety of pegylated-interferon α-2a in myelofibrosis: a study by the FIM and GEM French cooperative groups.
abstract: Myeloproliferative neoplasm-related myelofibrosis is associated with cytopenic or proliferative phases, splenomegaly and constitutional symptoms. Few effective treatments are available and small series suggested that interferon could be an option for myelofibrosis therapy. We performed a retrospective study of pegylated-interferon α-2a (Peg-IFNα-2a) therapy in myelofibrosis. Sixty-two patients treated with Peg-IFNα-2a at 17 French and Belgian centres were included. Responses were determined based on the criteria established by the International Working Group for Myelofibrosis Research and Treatment. Mean follow-up was 26 months. Sixteen of 25 anaemic patients (64%) (eight concomitantly receiving recombinant erythropoietin) achieved a complete response and transfusion-independence was obtained in 5/13 patients (38·5%). Constitutional symptoms resolved in 82% of patients. All five leucopenic patients normalized their leucocyte counts, whereas a normal platelet count was obtained in 5/8 thrombocytopenic patients. Splenomegaly was reduced in 46·5% of patients, and complete resolution of thrombocytosis and leucocytosis were observed in 82·8% and 68·8% of patients, respectively. Side effects (mostly haematological) were mainly of grade 1-2. The only factor independently associated with treatment failure was a spleen enlargement of more than 6 cm below the costal margin. In conclusion, Peg-IFNα-2a induced high response rates with acceptable toxicity in a large proportion of patients with primary and secondary myelofibrosis, especially in early phases.
authors.affiliation: Service d'hématologie clinique, Institut de Cancéro-Hématologie, CHRU Brest, Brest, France.
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Ianotto
authors.forename: Françoise
authors.initials: F
authors.lastname: Boyer-Perrard
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Gyan
authors.forename: Kamel
authors.initials: K
authors.lastname: Laribi
authors.forename: Pascale
authors.initials: P
authors.lastname: Cony-Makhoul
authors.forename: Jean-Loup
authors.initials: JL
authors.lastname: Demory
authors.forename: Benoit
authors.initials: B
authors.lastname: De Renzis
authors.forename: Christine
authors.initials: C
authors.lastname: Dosquet
authors.forename: Jerome
authors.initials: J
authors.lastname: Rey
authors.forename: Lydia
authors.initials: L
authors.lastname: Roy
authors.forename: Brigitte
authors.initials: B
authors.lastname: Dupriez
authors.forename: Laurent
authors.initials: L
authors.lastname: Knoops
authors.forename: Laurence
authors.initials: L
authors.lastname: Legros
authors.forename: Mohamed
authors.initials: M
authors.lastname: Malou
authors.forename: Pascal
authors.initials: P
authors.lastname: Hutin
authors.forename: Dana
authors.initials: D
authors.lastname: Ranta
authors.forename: Michele
authors.initials: M
authors.lastname: Schoenwald
authors.forename: Annalisa
authors.initials: A
authors.lastname: Andreoli
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Abgrall
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Kiladjian
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D006801:Humans; D016898:Interferon-alpha / Q000009:adverse effects* / Q000627:therapeutic use*; D008875:Middle Aged; D011092:Polyethylene Glycols / Q000009:adverse effects* / Q000627:therapeutic use*; D055728:Primary Myelofibrosis / Q000188:drug therapy* / Q000473:pathology; D011994:Recombinant Proteins / Q000009:adverse effects / Q000627:therapeutic use; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D016898:Interferon-alpha; D011994:Recombinant Proteins; D011092:Polyethylene Glycols; C100416:peginterferon alfa-2a
keywords: Myelofibrosis; interferon; myeloproliferative neoplasms