多细胞真血症:2024年关于诊断,风险分层和管理的更新
Ayalew Tefferi1, Tiziano Barbui2
1Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota, USA.
American journal of hematology
|June 26, 2023
概括
多细胞血症真 (PV) 是一种JAK2突变的新生体. 治疗重点是预防血栓形成,使用瘤切除术和阿司匹林,并为高危患者保留细胞减小疗法.
科学领域:
- 血液学 血液学 血液学
- 在瘤学瘤学.
- 遗传学 遗传学是一种遗传学.
背景情况:
- 多细胞血症真 (PV) 是一种JAK2突变的骨髓增殖性瘤,其特征是红细胞瘤,白细胞瘤,血栓细胞瘤,并增加了血栓形成,骨髓纤维化和急性骨髓性白血病的风险.
- 诊断包括识别JAK2突变与升高的血红蛋白/血红素水平,尽管骨髓检查可能会建议.
- 细胞遗传异常,如+9,染色体Y,+8和20q-的丧失,在15%-20%的患者中观察到.
研究的目的:
- 提供多细胞真菌病 (PV) 的全面概述,包括其诊断,遗传突变,预后和当前的治疗策略.
- 突出血栓形成的危险因素,并讨论复杂病例的具体管理考虑.
主要方法:
- 对包括JAK2突变和血红素水平在内的诊断标准的审查.
- 对细胞遗传异常和非JAK2突变 (例如,TET2,ASXL1) 的分析.
- 评估生存数据,风险分层和血栓形成预测因素.
主要成果:
- 平均存活时间约为15年,在40岁以下的患者中超过35年.
- 高风险因素包括老年,白细胞病,异常的型和不良突变.
- 血栓形成,PV后MF和AML的20年风险分别为约26%,16%和4%.
结论:
- 目前的治疗策略优先考虑血栓瘤预防和风险分层.
- 药物诱导的JAK2V617F等位基因负荷的减少不被认为是一种疾病修饰指标,除非伴有缓解.
- 对于怀孕,静脉血栓,,术后护理和PV后骨髓纤维化而言,存在特殊的管理考虑.
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