Objective: To investigate the value of myeloproliferative neoplasms Symptom Assessment Form total symptom score (MPN-SAF-TSS)in assessing constitutional symptoms among Ph/BCR- ABL negative myeloproliferative neoplasm (MPN)patients.
Methods: A cohort of 628 MPN patients were evaluated by MPN- SAF- TSS.
Results: Fatigue was the most common symptom (76.0%, 76.2%vs 89.9%)and the highest average severity of all the symptoms (3.46±2.97, 3.47±2.99vs 4.74±3.04 scores)among polycythemia vera (PV), essential thrombocythemia (ET)and primary myelofibrosis (PMF)patients. Using the MPN- SAF- TSS analysis, PMF patients showed highest burden of symptoms (28.9 ± 19.1), followed by PV patients (19.2 ± 16.8), and finally ET patients (17.1 ± 15.3). Instinct differences were observed between PMF and PV patients (χ(2)=6.371,P=0.021), PMF and ET patients (χ(2)= 14.020,P<0.001). No significant difference was found between PV and ET patients (χ(2)=2.281,P=0.191).
Conclusion: MPN- SAF- TSS was effective in evaluating the symptomatic burden among Ph/BCRABL negative MPN patients and could be used for serial assessment in this clinical setting.
目的: 评估Ph染色体和BCR-ABL阴性骨髓增殖性肿瘤（MPN）患者体质性症状负荷。
方法: 用骨髓增殖性肿瘤总症状评估量表（MPN-SAF-TSS）对628例MPN患者进行现场调查。
结果: 疲劳在真性红细胞增多症（PV）、原发性血小板增多症（ET）和原发性骨髓纤维化（PMF）患者中的症状负荷和发生率均最高[分别为（3.46±2.97）、（3.47±2.99）、（4.74±3.04）分和76.0%、76.2%、89.9%]。患者的总症状负荷由高到低依次为PMF、PV、ET[分别为（28.9±19.1）、（19.2±16.8）、（17.1±15.3）分]，PMF与PV、ET比较差异均有统计学意义（χ2=6.371，P=0.021；χ2=14.020，P<0.001），PV与ET之间差异无统计学意义（χ2=2.281，P=0.191）。
结论: MPN-SAF-TSS评估量表可有效评估MPN患者体质性症状，PV、ET和PMF患者中PMF的总症状负荷最高，用MPN-SAF-TSS评估量表进行症状评估应列为MPN治疗研究的评价指标。