[Health-related quality of life and its associated variables in Chinese patients with Philadelphia-negative myeloproliferative neoplasms].

Objectives: To explore health-related quality of life (HRQoL) and identify its associated variables in Chinese patients with Philadelphia-negative myeloproliferative neoplasms (MPNs) . Methods: In this cross-sectional study, anonymous questionnaires were distributed to adult patients with MPNs to assess symptom burden measured by MPN-10 and HRQoL measured by Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36) and the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 (EORTC QLQ-C30) . Results: The data from 1405 respondents with MPNs, including 645 (45.9%) with essential thrombocythemia (ET) , 297 (21.1%) with polycythemia vera (PV) , and 463 (33.0%) with myelofibrosis (MF) , were analyzed. 646 (46.0%) respondents were male. The median age was 56 (range, 18-99) years. The mean MPN-10 scores were 13.0±12.7, 15.0±14.7, and 21.0±16.6 (P<0.001) , and the physical component summary (PCS) and mental component summary (MCS) scores were 48.0±8.5, 47.0±9.0, and 42.0±10.0 (P<0.001) and 51.0±11.0, 50.0±10.8, and 49.0±11.1 (P=0.002) for respondents with ET, PV, and MF, respectively. Respondents with MF reported the lowest score of physical functioning, role functioning, emotional functioning, cognitive functioning, social function, and global health status (all P<0.01) and the highest score of fatigue, pain, dyspnea, appetite loss, diarrhea, and financial problems (all P<0.05) in EORTC QLQ-C30. Multivariate analyses revealed that higher MPN-10 scores were significantly associated with lower PCS (-0.220 to -0.277, P<0.001) and MCS (-0.244 to -0.329, P<0.001) scores; increasing age (-1.923 to -4.869; all P<0.05) , lower PCS score. Additionally, comorbidity (ies) , symptom at diagnosis, splenomegaly, anemia, unknown driver gene, and higher annual out-of-pocket cost were significantly associated with lower PCS and/or MCS scores. However, age ≥ 60 years, urban household registration, concomitant medication, and receiving ruxolitinib therapy in respondents with MF were associated with higher MCS scores. Weak correlations were found between MPN-10 score (except the subscale of appetite loss and constipation) and EORTC QLQ-C30 score in majority of subscales in respondents with ET (|r| = 0.193-0.457, all P<0.001) , PV (|r| = 0.192-0.529, all P<0.01) , and MF (|r| = 0.180-0.488, all P<0.001) , respectively. Conclusions: HRQoL in patients with MPN was significantly reduced, especially in patients with MF. Sociodemographic and clinical variables were significantly associated with the HRQoL in patients with MPNs.目的： 评估中国Ph阴性骨髓增殖性肿瘤（MPN）患者生活质量及其影响因素。 方法： 通过横断面研究，在全国范围内向成年MPN患者发放无记名调查问卷，采用骨髓增殖性肿瘤总症状评估量表（MPN-10）评估症状负荷，健康调查简表（SF-36）和欧洲癌症研究与治疗组织生活质量核心30问卷（EORTC QLQ-C30）量表评估生活质量。 结果： 在1405份可评估的调查者问卷中，血小板增多症（ET）、真性红细胞增多症（PV）、骨髓纤维化（MF）受访者分别为645例（45.9%）、297例（21.1%）、463例（33.0%），男性占46.0%（646例），中位年龄56（18~99）岁。ET、PV、MF受访者MPN-10量表评分分别为（13.0±12.7）、（15.0±14.7）、（21.0±16.6）分（P<0.001），SF-36量表躯体健康总评分（PCS）分别为（48.0±8.5）、（47.0±9.0）、（42.0±10.0）分（P<0.01），精神健康总评分（MCS）分别为（51.0±11.0）、（50.0±10.8）、（49.0±11.1）分（P=0.002）。EORTC QLQ-C30量表中，MF受访者躯体功能、角色功能、情绪功能、认知功能、社会功能和总体健康状况评分最低（P值均<0.05），疲劳、疼痛、呼吸困难、食欲丧失、腹泻和经济困难评分最高（P值均<0.05）。多因素分析结果显示，三种疾病中，MPN-10评分高（PCS：-0.220~-0.277，P值均<0.01；MCS：-0.244~-0.329，P值均<0.01）与MPN受访者PCS和MCS低显著相关，年龄增加（-1.923~-4.869，P值均<0.05）与PCS低显著相关。此外，共存疾病多、初诊时有症状、脾大、贫血、未知基因突变类型和自付治疗费用高与较低的PCS和（或）MCS相关。年龄≥60岁、城镇户籍、有合并用药、MF受访者采用芦可替尼治疗与较高的MCS相关。在ET、PV和MF受访者中，除食欲丧失和便秘外，MPN-10评分与EORTC QLQ-C30多呈低度相关（ET：|r|=0.193~0.457，P值均<0.01；PV：|r|=0.192~0.529，P值均<0.01；MF：|r|=0.180~0.488，P<0.001）。 结论： MPN患者生活质量受损，其中MF患者最差。社会人口学和临床因素显著影响MPN患者的生活质量，其中症状负荷是最重要的影响因素。.