142 例Ph染色体阴性的慢性骨髓增殖性肿瘤患者的体质性症状负荷评估

摘要

目的 研究Ph染色体阴性的慢性骨髓增殖性肿瘤(myeloproliferative neoplasms, MPNs)患者体质性症状负荷.方法 应用骨髓增殖性肿瘤总症状评分量表(MPN-SAF-TSS)对142 例MPNs患者进行随访调查.结果 MPN各亚型中PMF体质性症状的发生率最高,且与PV、ET相比差异均有统计学意义(χ2＝8.72,P﹦0.003;χ2＝14.063,P＜0.001),而PV与ET之间差异无统计学意义(χ2＝1.231,P＝0.267).各亚型总症状负荷由高到低依次为PMF[(20.67±18.38)分]、PV[(14.50±14.98)分]和ET[(6.78±9.33)分].ET的总症状负荷最低,与PV、PMF相比差异均有统计学意义(t＝－3.333, P＝0.001;t＝－5.028,P＜0.001),而PV与PMF之间差异无统计学意义(t＝－1.650,P＝0.103).其中,疲劳在PV、ET、PMF患者中的发生率和症状负荷均为最高.结论 MPN-SAF-TSS量表可有效评估MPN患者体质性症状,Ph阴性的MPNs患者中PMF体质性症状的发生率及总症状负荷最高,MPN-SAF-TSS评估量表进行症状评分可作为MPN治疗研究的评价指标.%Objective To investigate the constitutional symptoms burden among patients with Philadelphia chromosome negative myeloproliferative neoplasms (MPNs). Methods The constitutional symptoms burden of 142 MPN patients were evaluated by MPN-SAF-TSS. Results The highest incidence of constitutional symptoms was among primary myelofibrosis (PMF) patients, instinct differences were observed between PMF and polycythemia vera (PV) patients (χ2＝8.72, P＝0.003), PMF and essential thrombocythemia (ET) patients (χ2＝14.063, P＜0.001), while there was no statistically significant difference between PV and ET patients (χ2＝1.231, P＝0.267). PMF patients showed highest burden of symptoms (20.67±18.38), followed by PV patients (14.50±14.98), and finally ET patients (6.78±9.33). Compared with PV and PMF patients, ET patients showed the significant lower burden of symptoms (t＝－3.333, P＝0.001; t＝－5.028, P＜0.001), no significant difference was found between PV and PMF patients. Fatigue was the most common symptom and the highest burden of all the symptoms among PV, ET and PMF patients. Conclusion MPN-SAF-TSS could evaluate the constitutional symptoms burden among Ph negative MPN patients effectively, and can be used as one of indexes for assessment in the clinical setting. PMF patients showed the highest incidence and burden of constitutional symptoms.