关键词: CSF3R mutation Chronic eosinophilic leukemia Chronic neutrophilic leukemia World Health Organization Classification 5th edition

Mesh : Humans Leukemia, Neutrophilic, Chronic / genetics therapy complications Mutation Hypereosinophilic Syndrome / diagnosis therapy complications Leukemia, Myeloid / complications

来  源:   DOI:10.11406/rinketsu.64.1326

Abstract:
Chronic neutrophilic leukemia (CNL) is a clonal disorder that is characterized by increasing mature neutrophils. Colony stimulating factor 3 receptor (CSF3R) T618I mutation was frequently identified in patients with CNL and is defined as a molecular marker of the disease. Ruxolitinib, a JAK2 inhibitor, provided a promising therapeutic effect in a phase II study. In particular, ruxolitinib was more efficient for patients with CSF3R mutation. Allogeneic stem cell transplantation (Allo-SCT) may be a curative treatment for CNL. On the other hand, further studies are needed to define the optimal method of transplantation, source of donor, conditioning therapy, and timing of transplantation. Chronic eosinophilic leukemia (CEL) is a clonal disorder that is characterized by increasing eosinophils. In the World Health Organization Classification 5th edition, diagnostic criteria for CEL are renewed. Because the new criteria will be more specific for CEL than criteria in the older edition, \"not otherwise specified (NOS) \" is removed from the name of the disease. Anti-CD52 antibody, alemtuzumab, or anti-IL-5 antibody, mepolizumab, are promising drugs to control symptoms that are associated with hypereosinophilic syndrome. Allo-SCT is anticipated as a curative treatment for CEL, but the evidence of Allo-SCT for CEL is still limited. Further study is required to define the treatment strategy.
摘要:
慢性嗜中性粒细胞白血病(CNL)是一种克隆性疾病,其特征是成熟的嗜中性粒细胞增多。集落刺激因子3受体(CSF3R)T618I突变在CNL患者中经常被发现,并被定义为该疾病的分子标志物。鲁索替尼,JAK2抑制剂,在II期研究中提供了有希望的治疗效果。特别是,ruxolitinib对CSF3R突变患者更有效.同种异体干细胞移植(Allo-SCT)可能是CNL的治愈性治疗。另一方面,需要进一步的研究来确定最佳的移植方法,捐赠者的来源,调理疗法,和移植的时机。慢性嗜酸性粒细胞白血病(CEL)是一种以嗜酸性粒细胞增多为特征的克隆性疾病。在世界卫生组织分类第5版中,CEL的诊断标准更新。因为新标准将比旧版本的标准更具体,从疾病名称中删除“未指定(NOS)”。抗CD52抗体,阿仑单抗,或抗IL-5抗体,美波利单抗,是控制与嗜酸性粒细胞增多综合征相关症状的有前途的药物。Allo-SCT有望作为CEL的治愈性治疗,但是用于CEL的Allo-SCT的证据仍然有限。需要进一步的研究来确定治疗策略。
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