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Prognostic Impact of JAK2V617F Mutation In MDS: a Matched Case Control Study

Authors :
Gérard Tertian
Romain Guieze
Florence Nguyen-Khac
Olivier Tournilhac
Lionel Ades
Jean-Loup Demory
Dominique Bordessoule
Françoise Boyer
Odile Beyne-Rauzy
Christian Rose
Eric Wattel
Michaela Fontenay
Jean-Jacques Kiladjian
Jean Christophe Ianotto
Pierre Fenaux
Aurélie Cabrespine
Benoit de Renzis
Sophie Raynaud
Laurent Knoops
Jean-Yves Cahn
Source :
Blood. 116:440-440
Publication Year :
2010
Publisher :
American Society of Hematology, 2010.

Abstract

Abstract 440 Background: The JAK2V617F mutation is found in a small proportion of MDS, especially in RARS-T and occasionally in other MDS subtypes, but the overall impact of JAK2V617F on MDS characteristics and outcome remains unclear. Method: Diagnostic and follow up data on MDS patients (pts) with known JAK2V617F mutation status were collected from 19 centers of the Groupe Francophone des Myélodysplasies (GFM) and the French Intergroup of MPN (FIM). MDS post MPN and CMML were excluded. Patient characteristics and outcome according to JAK2V617F status were analyzed by univariate analysis. Survival analysis with Cox model matched on age, IPSS score and sex according to JAK2 status was also made. Analysis was performed using STATA 10.0 software. Result: 161 cases were collected, including 65 JAK2V617F mutated (JAK2 pos) and 96 unmutated (JAK2 neg) cases. Median age was 75 years and M/F ratio 1.2 in JAK2 pos vs 71 years (p=NS) and 1 (p=NS) in JAK2 neg pts, respectively (resp). WHO 2008 distribution was RA (8%), RARS (12%), RARS-T (41%), CRDM (15%), RAEB-1 (11%), RAEB-2 (5%), 5q- (3%), unclassified (5%) in JAK2 pos pts and RA (25%), RARS (9%), RARS-T (1%), CRDM (14%), RAEB-1 (28%), RAEB-2 (19%), 5q- (1%), unclassified (3%) in JAK2 neg pts, resp (p Conclusion: We found JAK2V617F mutation in MDS to be associated with higher WBC, ANC and platelet counts, lower % marrow blasts, less progression to AML and better survival than JAK2V617F neg MDS. This positive prognostic impact persisted after exclusion of RARS-T. However, our results will require confirmation in a prospective study. Disclosures: Fenaux: CELGENE, JANSSEN CILAG, AMGEN, ROCHE, GSK, NOVARTIS, MERCK, CEPHALON: Honoraria, Research Funding.

Details

ISSN :
15280020 and 00064971
Volume :
116
Database :
OpenAIRE
Journal :
Blood
Accession number :
edsair.doi...........8d8048a8164f5aecfed0b82bdc94db89
Full Text :
https://doi.org/10.1182/blood.v116.21.440.440