SEARCH:   Advanced

Blood, 20 August 2009, Vol. 114, No. 8, pp. 1477-1483. Prepublished online as a Blood First Edition Paper on June 23, 2009; DOI 10.1182/blood-2009-04-216044. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2009-04-216044v1 114/8/1477    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via CrossRef Google Scholar Articles by Guglielmelli, P. Articles by Vannucchi, A. M. PubMed PubMed Citation Articles by Guglielmelli, P. Articles by Vannucchi, A. M. Related Collections Free Research Articles Myeloid Neoplasia Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL TRIALS AND OBSERVATIONS Identification of patients with poorer survival in primary myelofibrosis based on the burden of JAK2V617F mutated allele Paola Guglielmelli1, Giovanni Barosi2, Giorgina Specchia3, Alessandro Rambaldi4, Francesco Lo Coco5, Elisabetta Antonioli1, Lisa Pieri1, Alessandro Pancrazzi1, Vanessa Ponziani1, Federica Delaini4, Giovanni Longo1, Emanuele Ammatuna5, Vincenzo Liso3, Alberto Bosi1, Tiziano Barbui4, and Alessandro M. Vannucchi1 1 Unità Funzionale di Ematologia, Dipartimento di Area Critica, Università di Firenze, and Istituto Toscano Tumori, Firenze; 2 Unità di Epidemiologia Clinica, Fondazione Istituti di Ricovero e Cura a Carattere Scientifico, Policlinico S Matteo, Pavia; 3 Dipartimento di Ematologia, Università di Bari, Bari; 4 Divisione di Ematologia, Ospedali Riuniti, Bergamo; and 5 Dipartimento di Biopatologia e Diagnostica per Immagini, Università Tor Vergata, Rome, Italy A total of 186 patients with primary myelofibrosis (PMF) were genotyped for JAK2V617F at diagnosis aimed at analyzing the correlation of mutational status and mutated allele burden with outcome variables, including time to anemia, leukocytosis, leukopenia, thrombocytopenia, massive splenomegaly, leukemia, and with overall survival. A total of 127 JAK2V617F-mutated patients (68% of whole series) were divided in quartiles of V617F allele burden. After a median follow-up of 17.2 months, 23 patients died, 15 because of leukemia. A JAK2V617F mutated status did not impact on the rate of leukemia transformation or overall survival. Patients in the lower quartile had shorter time to anemia and leukopenia and did not progress to large splenomegaly. Furthermore, survival was significantly reduced in the lower quartile compared with upper quartiles and JAK2 wild-type patients. In multivariate analysis, factors associated with reduced survival were age, a blast count more than 1%, and a JAK2V617F burden within first quartile. Causes of death in the lower quartile were represented mainly by systemic infections. We conclude that a low JAK2V617F allele burden at diagnosis is preferentially associated with a myelodepletive rather than myeloproliferative phenotype and represents an independent factor associated with shortened survival in patients with PMF. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

	Copyright © 2009 by American Society of Hematology         Online ISSN: 1528-0020