Itzykson Raphael, Fenaux Pierre, Bowen David, Cross Nicholas C P, Cortes Jorge, De Witte Theo, Germing Ulrich, Onida Francesco, Padron Eric, Platzbecker Uwe, Santini Valeria, Sanz Guillermo F, Solary Eric, Van de Loosdrecht Arjan, Malcovati Luca
Department of Hematology, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Paris Diderot University, Paris, France.
St. James's Institute of Oncology, Leeds Teaching Hospitals, Leeds, United Kingdom.
Hemasphere. 2018 Nov 29;2(6):e150. doi: 10.1097/HS9.0000000000000150. eCollection 2018 Dec.
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, we present 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.
慢性粒单核细胞白血病(CMML)是一种老年疾病,是目前成人中最常见的重叠型骨髓增生异常/骨髓增殖性肿瘤。除了作为其诊断基石的慢性单核细胞增多外,CMML的临床表现还包括发育异常特征、血细胞减少、原始细胞增多,或骨髓增殖性特征,如白细胞计数升高或脾肿大。预后各不相同,近年来报道了几种预后评分系统,治疗方法尚不明确,选择范围从观察等待到异基因干细胞移植,而异基因干细胞移植仍然是CMML唯一的治愈性疗法。在此,我们代表欧洲血液学协会和欧洲白血病网,展示由来自欧洲和美国的国际专家小组制定的基于证据和共识的指南,用于成人CMML患者标准化的诊断和预后程序以及适当的治疗干预选择。