Clinical features and course of refractory anemia with ring sideroblasts associated with marked thrombocytosis
Clinical features and course of refractory anemia with ring sideroblasts associated with marked thrombocytosis
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DOI：
10.3324/haematol.2011.053918
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发表时间：
2012-07-01
期刊：
影响因子：
--
通讯作者：
Girodon, Francois
中科院分区：
文献类型：
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作者：
Broseus, Julien;Florensa, Lourdes;Girodon, Francois
环状铁粒细胞伴发明显的血小板增多症的难治性贫血在2001年世界卫生组织对髓系肿瘤的分类中被认为是一个临时实体，在2008年的版本中也是如此，但它作为一个单一实体的存在存在争议。我们希望确定这种罕见的骨髓增生异常/骨髓增生性肿瘤的临床特征，并将其临床结果与环状铁粒母细胞和原发性血小板增多症的难治性贫血进行比较。设计与方法我们在整个欧洲进行了一项协作性的回顾性研究。我们的数据库包括200名诊断为环状铁粒细胞并有明显的血小板增多的顽固性贫血患者。对于这些患者中的每一位，每一位被诊断为环状铁粒细胞的顽固性贫血患者在年龄和性别上都是匹配的。结果在有环状铁粒细胞和明显血小板增多症的难治性贫血患者中，根据Janus Kinase 2 V617F突变状态(阳性或阴性)或血小板阈值(高于或低于600x10(9)/L)的不同，患者的生存率没有差异。然而，与原发性血小板增多症患者相比，这些患者的总生存期和无白血病生存期更短，血栓并发症的风险更低(P
BackgroundRefractory 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 MethodsWe 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.ResultsIn 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