Please use this identifier to cite or link to this item: http://hdl.handle.net/2445/126519
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
Luno, Elisa
Pinan, Maria Angeles
Arenillas, 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
Related resource: https://doi.org/10.3324/haematol.2011.053918
URI: http://hdl.handle.net/2445/126519
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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