Clinical features and course of refractory anemia with ring sideroblasts associated with marked thrombocytosis

Julien Broseus, Lourdes Florensa, Esther Zipperer, Susanne Schnittger, Luca Malcovati, Steven Richebourg, Eric Lippert, Jaroslav Cermak, Jyoti Evans, Morgane Mounier, José Maria Raya, François Bailly, Norbert Gattermann, Torsten Haferlach, Richard Garand, Kaoutar Allou, Carlos Besses, Ulrich Germing, Claudia Haferlach, Erica TravaglinoElisa Luno, Maria Angeles Pinan, Leonor Arenillas, Maria Rozman, Maria Luz Perez Sirvent, Bernardine Favre, Julien Guy, Esther Alonso, Nuhri Ahwij, Andrés Jerez, Sylvie Hermouet, Marc Maynadié, Mario Cazzola, François Girodon

Research output: Contribution to journalArticlepeer-review

Abstract

Background Refractory anemia with ring sideroblasts associated with marked thrombocytosis was proposed as a provisional entity in the 2001 World Health Organization classification of myeloid neoplasms and also in the 2008 version, but its existence as a single entity is contested. We wish to define the clinical features of this rare myelodysplastic/myeloproliferative neoplasm and to compare its clinical outcome with that of refractory anemia with ring sideroblasts and essential thrombocythemia. Design and Methods We conducted a collaborative retrospective study across Europe. Our database included 200 patients diagnosed with refractory anemia with ring sideroblasts and marked thrombocytosis. For each of these patients, each patient diagnosed with refractory anemia with ring sideroblasts was matched for age and sex. At the same time, a cohort of 454 patients with essential thrombocythemia was used to compare outcomes of the two diseases. Results In patients with refractory anemia with ring sideroblasts and marked thrombocytosis, depending on the Janus Kinase 2 V617F mutational status (positive or negative) or platelet threshold (over or below 600×109/L), no difference in survival was noted. However, these patients had shorter overall survival and leukemia-free survival with a lower risk of thrombotic complications than did patients with essential thrombocythemia (P

Original languageEnglish
Pages (from-to)1036-1041
Number of pages6
JournalHaematologica
Volume97
Issue number7
DOIs
Publication statusPublished - Jul 1 2012

Keywords

  • Clinical course
  • Essential thrombocythemia
  • Refractory anemia
  • Ring sideroblasts
  • Thrombocytosis

ASJC Scopus subject areas

  • Hematology

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