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Title: | Clinical Features And Course Of Refractory Anemia With Ring Sideroblasts Associated With Marked Thrombocytosis |
Author: | Broseus, Julien Florensa, Lourdes Zipperer, Esther Schnittger, Susanne Malcovati, Luca Richebourg, Steven Lippert, Eric Cermak, Jaroslav Evans, Jyoti Mounier, Morgane Raya, José Maria Bailly, François Gattermann, Norbert Haferlach, Torsten Garand, Richard Allou, Kaoutar Besses, Carlos Germing, Kaoutar Haferlach, Claudia Travaglino, Erica Luño, Elisa Pinan, Maria Angeles Arenillas Rocha, Leonor Rozman, María Pérez Sirvent, Maria Luz Favre, Bernardine Guy, Julien Alonso Sanz, Esther Ahwij, Nuhri Jerez, Andrés Hermouet, Sylvie Maynadié, Marc Cazzola, Mario Girodon, François |
Keywords: | Anèmia Tumors Anemia |
Issue Date: | Jul-2012 |
Publisher: | Ferrata Storti Foundation |
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 600x10(9)/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<0.001) but better survival (P<0.001) and a higher risk of thrombosis (P=0.039) than patients with refractory anemia with ring sideroblasts. Conclusions: The clinical course of refractory anemia with ring sideroblasts and marked thrombocytosis is better than that of refractory anemia with ring sideroblasts and worse than that of essential thrombocythemia. The higher risk of thrombotic events in this disorder suggests that anti-platelet therapy might be considered in this subset of patients. From a clinical point of view, it appears to be important to consider refractory anemia with ring sideroblasts and marked thrombocytosis as a distinct entity. |
Note: | Reproducció del document publicat a: https://doi.org/10.3324/haematol.2011.053918 |
It is part of: | Haematologica, 2012, vol. 97, num. 7, p. 1036-1041 |
URI: | http://hdl.handle.net/2445/126519 |
Related resource: | https://doi.org/10.3324/haematol.2011.053918 |
Appears in Collections: | Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer) |
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