Belfast City Hospital, Belfast Trust, Belfast, Northern Ireland.
Ninewells Hospital and Medical School, NHS Tayside, Dundee, Scotland.
Ir J Med Sci. 2024 Apr;193(2):833-836. doi: 10.1007/s11845-023-03510-7. Epub 2023 Sep 8.
Polycythaemia vera (PV) is a Philadelphia-negative myeloproliferative neoplasm, typically driven by acquired JAK2 mutation and characterised by elevated red cell mass and increased risk of thrombotic events. Patients are managed with phlebotomy to maintain haematocrit (Hct) < 0.45, and patients stratified as 'high risk' for thrombosis are additionally treated with cytoreductive agents to attain this target.
This analysis of newly diagnosed JAK2 mutant PV patients (n = 50) over 2 years aimed to determine how effectively patients attained and maintained target Hct according to recommended practice.
We found that patients spent the majority of time in target Hct range. Findings are supportive of current management guidelines.
真性红细胞增多症(PV)是一种费城阴性骨髓增殖性肿瘤,通常由获得性 JAK2 突变驱动,其特征是红细胞质量增加,血栓形成事件的风险增加。患者通过放血治疗来维持血细胞比容(Hct)<0.45,并且被分层为血栓形成“高危”的患者还需要用细胞毒性药物来达到这一目标。
本研究对新诊断的 JAK2 突变 PV 患者(n=50)进行了为期 2 年的分析,旨在根据推荐的实践确定患者达到和维持目标 Hct 的效果如何。
我们发现患者在目标 Hct 范围内的时间居多。这些发现支持当前的管理指南。