615例PH染色体/BCR-ABL融合基因阴性骨髓增殖性肿瘤患者的症状负荷评估

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±2.99 vs 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.