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Semin Thromb Hemost 2006; 32(4): 417-421
DOI: 10.1055/s-2006-942762
Copyright © 2006 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Long-Term Incidence of Hematological Evolution in Three French Prospective Studies of Hydroxyurea and Pipobroman in Polycythemia Vera and Essential Thrombocythemia

Jean-Jacques Kiladjian1 , Jean-Didier Rain2 , Jean-Francois Bernard1 , Jean Briere1 , Christine Chomienne2 , Pierre Fenaux1
  • 1AP-HP, Hopital Avicenne, Service d'Hematologie Clinique; Universite Paris 13; Bobigny, France
  • 2AP-HP, Hopital Saint-Louis, Unite de Biologie Cellulaire Paris, France
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Publication History

Publication Date:
29 June 2006 (online)

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ABSTRACT

Despite recent discoveries made in myeloproliferative disorders other than chronic myelogenous leukemia, which it is hoped will result in earlier diagnosis, and better evaluation and management of patients, hematological evolution to myelofibrosis, acute leukemia, and myelodysplastic syndromes (AL/MDS) remain major causes of long-term mortality in polycythemia vera (PV) and essential thrombocythemia (ET) patients. Evaluation of long-term leukemogenic risk of currently available drugs, therefore, is crucial. We report updated results of three French prospective trials of hydroxyurea and pipobroman in PV and ET patients with a median follow-up longer than 10 years. The results show that the incidence of AL/MDS is higher than previously reported with no evidence of a plateau (with ~40% of AL/MDS cases occurring after the 12th year of follow-up). Although hydroxyurea currently remains the first choice in the treatment of high-risk PV and ET patients, the use of nonleukemogenic drugs, such as interferon α (IFN-α) or anagrelide, should be assessed more widely in randomized trials using accurate diagnostic criteria and taking into account the presence of the JAK2 mutation, given that they may have an impact on disease evolution.

KEYWORDS

Polycythemia vera - essential thrombocythemia - leukemogenic risk - hydroxyurea - anagrelide - interferon - pipobroman

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Jean-Jacques KiladjianM.D. 

Hopital Avicenne and Paris 13 University, Service d'Hematologie clinique

125, route de Stalingrad, 93000 Bobigny, France

Email: jean-jacques,kiladjian@avc.aphp.fr

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