Department of Hematology, Medical Science University İstanbul Prof. Dr. Cemil Taşcioğlu City Hospital, İstanbul, Türkiye.
Department of Hematology, Ege University Faculty of Medicine, İzmir, Türkiye.
Medicine (Baltimore). 2024 Jul 26;103(30):e38814. doi: 10.1097/MD.0000000000038814.
Previous studies have shown that patients with polycythemia vera (PV) have poor quality of life (QoL). Similarly, it has been shown that survival is influenced by QoL. We aimed to evaluate QoL in 88 Turkish patients with PV. This cross-sectional study included cases diagnosed with PV between January 1995 and August 2019 who attended follow-up studies in the hematology department of a tertiary hospital in Türkiye between August 2019 and July 2020. Beginning in August 2019, subjects who approved study participation applied the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire (EORTC QLQ-C30) questionnaire during their routine follow-up-given that they met inclusion/exclusion criteria. Individuals with comorbidities or factors influencing QoL and those with secondary PV-related conditions were excluded. Recorded data included age, sex, history of bleeding, thrombosis, erythrocytosis, leukocytosis, thrombocytosis, obesity or splenomegaly, and cytogenetic mutation profiles such as JAK2, BCR and MPL. We also assessed whether they needed phlebotomy or erythrocyte suspensions. Data concerning comorbidities and medication use were obtained from medical records. The median age of patients was 52 (44-61) years. The majority of participants were male (67.05%). Global health status score was 75 (66.67-83.33). PV patients who had required phlebotomy demonstrated higher social functioning scores (P = .004) and lower scores for loss of appetite (P = .013) and financial difficulties (P = .020) than patients without phlebotomy. PV patients who had suffered from leukocytosis demonstrated lower physical functioning scores compared to those without leukocytosis (P = .001). Patients without JAK2 exon 14 mutations had better physical (P = .016) and cognitive functioning scores (P = .048). It was found that PV patients with splenomegaly demonstrated lower physical functioning (P = .019) and higher appetite loss scores (P = .005) than those without splenomegaly. Higher leucocyte counts were associated with decreased physical functioning and greater fatigue. In conclusion, we demonstrated deterioration of physical and emotional QoL in patients diagnosed with PV. Patients with PV require individualized, patient-specific and integrated approaches in order to minimize symptoms, improve QoL, and increase survival.
先前的研究表明,真性红细胞增多症 (PV) 患者的生活质量 (QoL) 较差。同样,研究表明生存受到 QoL 的影响。我们旨在评估 88 名土耳其 PV 患者的 QoL。这项横断面研究包括 1995 年 1 月至 2019 年 8 月期间在土耳其一家三级医院血液科就诊并于 2019 年 8 月至 2020 年 7 月期间接受常规随访的确诊为 PV 的病例。自 2019 年 8 月起,符合纳入/排除标准的符合条件的患者在常规随访期间开始使用欧洲癌症研究与治疗组织生活质量问卷 (EORTC QLQ-C30) 进行问卷调查。患有合并症或影响 QoL 的因素以及患有继发性 PV 相关疾病的患者被排除在外。记录的数据包括年龄、性别、出血史、血栓形成史、红细胞增多症、白细胞增多症、血小板增多症、肥胖或脾肿大以及 JAK2、BCR 和 MPL 等细胞遗传学突变谱。我们还评估了他们是否需要放血或红细胞悬浮液。合并症和药物使用的数据从病历中获得。患者的中位年龄为 52 岁(44-61 岁)。大多数参与者为男性(67.05%)。总体健康状况评分为 75 分(66.67-83.33)。需要放血的 PV 患者的社会功能评分较高(P=0.004),食欲丧失评分(P=0.013)和经济困难评分(P=0.020)较低。与无白细胞增多症的患者相比,白细胞增多症患者的身体功能评分较低(P=0.001)。无 JAK2 外显子 14 突变的患者的身体(P=0.016)和认知功能评分(P=0.048)更好。脾肿大的 PV 患者的身体功能(P=0.019)和食欲丧失评分(P=0.005)较高。白细胞计数较高与身体功能下降和疲劳增加有关。总之,我们发现诊断为 PV 的患者的身体和情绪 QoL 恶化。PV 患者需要个体化、针对患者的综合方法,以最小化症状、改善 QoL 和提高生存率。