Page 78 - Haematologica3

Correspondence:
jean-jacques.kiladjian@aphp.fr
Received: September 22, 2017. Accepted: December 6, 2017. Pre-published: December 7, 2017.
doi:10.3324/haematol.2017.181297
Check the online version for the most updated information on this article, online supplements, and information on authorship & disclosures: www.haematologica.org/content/103/3/438
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Haematologica 2018 Volume 103(3):438-446
Jean-Christophe Ianotto,1* Aurélie Chauveau,2* Françoise Boyer-Perrard,3 Emmanuel Gyan,4 Kamel Laribi,5 Pascale Cony-Makhoul,6 Jean-Loup Demory,7 Benoit de Renzis,8 Christine Dosquet,9 Jerome Rey,10 Lydia Roy,11 Brigitte Dupriez,12 Laurent Knoops,13 Laurence Legros,14 Mohamed Malou,15 Pascal Hutin,16 Dana Ranta,17 Omar Benbrahim,18 Valérie Ugo,19 Eric Lippert2** and Jean-Jacques Kiladjian20**
Ferrata Storti Foundation
Myeloproliferative Neoplasms
Benefits and pitfalls of pegylated interferon-α2a therapy in patients with myeloproliferative neoplasm-associated myelofibrosis:
a French Intergroup of Myeloproliferative neoplasms (FIM) study
1Service d’Hématologie Clinique, Institut de Cancéro-hématologie, CHRU Brest, France; 2Laboratoire d’Hématologie, CHRU de Brest and INSERM U1078, Université de Bretagne Occidentale, Brest, France; 3Service des Maladies du Sang, CHU d’Angers, France; 4Hématologie et Thérapie Cellulaire, CRU de Cancérologie H.S. Kaplan, Tours, France; 5Service d’Hématologie, CH Le Mans, France; 6Service d’Hématologie, CH Annecy- Genevois, France; 7Service d’Hématologie, Hôpital St Vincent de Paul, Lille, France; 8Service d’Hématologie, CHU Clermont-Ferrand, France; 9APHP, Saint Louis Hospital, Cell Biology Department, Paris, France; 10Département d’Hématologie, Institut Paoli-Calmette, Marseille, France; 11Service d’Hématologie, Hôpital de Créteil, France; 12Service d’Hématologie Clinique, CHU de Lens, France; 13Cliniques Universitaires Saint-Luc and Université Catholique de Louvain, Brussels, Belgium; 14Service d’Hématologie, CHU de Nice, France; 15Service d’Oncologie et D’Hématologie, Hôpital de Morlaix, France; 16Service de Médecine Interne et de Maladies Infectieuses, Hôpital Laennec, Quimper, France; 17Département d’Hématologie, Hôpital Universitaire de Nancy, Vandœuvre-lès-Nancy, France; 18Service d’Hématologie, Hôpital La Source, Orléans, France; 19Laboratoire d’Hématologie, CHU d’Angers, France and 20Centre d’Investigation Clinique, Hôpital Saint- Louis, APHP, Université Paris Diderot, Inserm, Paris, France
*JCI and AC contributed equally to this manuscript. **EL and JJK contributed equally to this manuscript.
438
ABSTRACT
We have previously described the safety and efficacy of pegy- lated interferon-α2a therapy in a cohort of 62 patients with myeloproliferative neoplasm-associated myelofibrosis fol- lowed in centers affiliated to the French Intergroup of Myeloproliferative neoplasms. In this study, we report their long-term outcomes and correlations with mutational patterns of driver and non- driver mutations analyzed by targeted next generation sequencing. The median age at diagnosis was 66 years old, the median follow-up since starting pegylated interferon was 58 months. At the time of analysis, 30 (48.4%) patients were alive including 16 still being treated with pegylated interferon. The median survival of patients with inter- mediate and high-risk prognostic Lille and dynamic International Prognostic Scoring System scores treated with pegylated interferon was increased in comparison to that of historical cohorts. In addition, overall survival was significantly correlated with the duration of pegy- lated interferon therapy (70 versus 30 months after 2 years of treat- ment, P<10-12). JAK2V617F allele burden was decreased by more than 50% in 58.8% of patients and two patients even achieved complete molec- ular response. Next-generation sequencing analyses performed in 49 patients showed that 28 (57.1%) of them carried non-driver mutations. The presence of at least one additional mutation was associated with a reduction of both overall and leukemia-free survival. These findings in a large series of patients with myelofibrosis suggest that pegylated
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