Hematology - Bone Marrow Transplantation Center, Fondazione IRCCS Ospedale Maggiore Policlinico, University of Milan, Milan, Italy.
Haematologica. 2013 Sep;98(9):1344-52. doi: 10.3324/haematol.2013.084020.
With the aim of reviewing critical concepts and producing recommendations for the management of chronic myelomonocytic leukemia, key questions were selected according to the criterion of clinical relevance. Recommendations were produced using a Delphi process and four consensus conferences involving a panel of experts appointed by the Italian Society of Hematology and affiliated societies. This report presents the final statements and recommendations, covering patient evaluation at diagnosis, diagnostic criteria, risk classification, first-line therapy, monitoring, second-line therapy and allogeneic stem cell transplantation. For the first-line therapy, the panel recommended that patients with myelodysplastic-type chronic myelomonocytic leukemia and less than 10% blasts in bone marrow should be managed with supportive therapy aimed at correcting cytopenias. In patients with myelodysplastic-type chronic myelomonocytic leukemia with a high number of blasts in bone marrow (≥ 10%), supportive therapy should be integrated with the use of 5-azacytidine. Patients with myeloproliferative-type chronic myelomonocytic leukemia with a low number of blasts (<10%) should be treated with cytoreductive therapy. Hydroxyurea is the drug of choice to control cell proliferation and to reduce organomegaly. Patients with myeloproliferative-type chronic myelomonocytic leukemia, and a high number of blasts should receive polychemotherapy. Both in myelodysplastic-type and myeloproliferative-type chronic myelomonocytic leukemia, allogeneic stem cell transplantation should be offered within clinical trials in selected patients.
为了回顾慢性粒单核细胞白血病的关键概念并提出管理建议，根据临床相关性标准选择了关键问题。使用 Delphi 流程和涉及意大利血液学会及其附属学会任命的专家小组的四次共识会议制定了建议。本报告介绍了最终的陈述和建议，涵盖了诊断时的患者评估、诊断标准、风险分类、一线治疗、监测、二线治疗和异基因造血干细胞移植。对于一线治疗，专家组建议骨髓中具有骨髓增生异常型慢性粒单核细胞白血病和小于 10%原始细胞的患者应接受支持性治疗以纠正细胞减少症。骨髓中具有高数量原始细胞（≥ 10%）的骨髓增生异常型慢性粒单核细胞白血病患者应将支持性治疗与 5-氮杂胞苷联合使用。骨髓中具有低数量原始细胞（<10%）的骨髓增生性慢性粒单核细胞白血病患者应接受细胞减少性治疗。羟基脲是控制细胞增殖和减少器官肿大的首选药物。骨髓增生性慢性粒单核细胞白血病患者，具有高数量原始细胞的患者应接受多化疗。在骨髓增生异常型和骨髓增生性慢性粒单核细胞白血病中，均应在临床试验中为选定患者提供异基因造血干细胞移植。
