Diagnosis and Treatment of Chronic Myelomonocytic Leukemias in Adults Recommendations From the European Hematology Association and the European LeukemiaNet
Diagnosis and Treatment of Chronic Myelomonocytic Leukemias in Adults Recommendations From the European Hematology Association and the European LeukemiaNet
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DOI：
10.1097/hs9.0000000000000150
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发表时间：
2018-12-01
期刊：
影响因子：
6.6
通讯作者：
Malcovati, Luca
中科院分区：
文献类型：
--
作者：
Itzykson, Raphael;Fenaux, Pierre;Malcovati, Luca
慢性粒单核细胞白血病（CMML）是一种老年人疾病，也是迄今为止成人中最常见的重叠骨髓增生异常/骨髓增生性肿瘤。除了作为诊断基础的慢性单核细胞增多症外，CMML的临床表现还包括发育异常特征、血细胞减少、原始细胞过多或骨髓增生特征，包括高白色血细胞计数或脾肿大。预后是可变的，近年来报道了几种预后评分系统，治疗定义不清，选择范围从观察等待到异基因干细胞移植，这仍然是CMML的唯一治愈性治疗。在此，我们代表欧洲血液学协会和欧洲白血病网，提出了一个基于证据和共识的指南，该指南由来自欧洲和美国的国际专家组制定，用于标准化诊断和预后程序以及CMML成人患者治疗干预的适当选择。
Chronic myelomonocytic leukemia (CMML) is a disease of the elderly, and by far the most frequent overlap myelodysplastic/myeloproliferative neoplasm in adults. Aside from the chronic monocytosis that remains the cornerstone of its diagnosis, the clinical presentation of CMML includes dysplastic features, cytopenias, excess of blasts, or myeloproliferative features including high white blood cell count or splenomegaly. Prognosis is variable, with several prognostic scoring systems reported in recent years, and treatment is poorly defined, with options ranging from watchful waiting to allogeneic stem cell transplantation, which remains the only curative therapy for CMML. Here, wepresent on behalf of the European Hematology Association and the European LeukemiaNet, evidence- and consensus-based guidelines, established by an international group of experts, from Europe and the United States, for standardized diagnostic and prognostic procedures and for an appropriate choice of therapeutic interventions in adult patients with CMML.