6533b82ffe1ef96bd1295299
RESEARCH PRODUCT
Clinical Features And Course Of Refractory Anemia With Ring Sideroblasts Associated With Marked Thrombocytosis
Erica TravaglinoMaría ÁNgeles PiñanNorbert GattermannFrançois GirodonLeonor ArenillasKaoutar AllouFrançois BaillyMaria Luz Perez SirventMaría RozmanUlrich GermingBernardine FavreEsther ZippererJulien BroséusMario CazzolaCarlos BessesLuca MalcovatiJosé María RayaMarc MaynadiéEsther AlonsoJyoti EvansAndres JerezTorsten HaferlachMorgane MounierSteven RichebourgElisa LuñoEric LippertClaudia HaferlachJaroslav CermakRichard GarandSylvie HermouetNuhri AhwijJulien GuySusanne SchnittgerLourdes Florensasubject
AdultBlood PlateletsMalemedicine.medical_specialtyAdolescentAnemiaAnèmiaRefractory anemia with ringed sideroblastsLower riskGastroenterologyRisk FactorsInternal medicinehemic and lymphatic diseasesmedicineHumansMyeloproliferative neoplasmSurvival analysisAgedRetrospective StudiesTumorsAged 80 and overThrombocytosisThrombocytosisPlatelet CountEssential thrombocythemiabusiness.industryAnemia RefractoryAnemiaHematologyJanus Kinase 2Middle Agedmedicine.diseaseSurvival AnalysisAnemia SideroblasticSurgeryEuropeRefractory anemia with ring sideroblastsMutationFemaleOriginal Articles and Brief ReportsbusinessThrombocythemia Essentialdescription
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 <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.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2012-01-01 |