■重型β-地中海贫血(β-TM)是全球最重要的血红蛋白病之一。在基于输血和铁螯合的支持治疗中取得了显著的改善,如今,这种方法能够确保工业化国家的这些患者的长寿。唯一的治愈性治疗以造血干细胞移植(HSCT)为代表。然而,健康相关生活质量(HRQoL)恶化可能会给这种治疗带来负担.本文旨在通过系统评价和荟萃分析评价HRQoL在移植β-TM患者中的作用。
■PubMed数据库,WebofScience,和Scopus被系统地搜索了1月1日之间发表的研究,2000年至2020年9月。在数据库查询中输入了以下术语:β-地中海贫血,HRQoL,和HSCT。根据系统和荟萃分析的首选报告项目(PRISMA)声明进行研究。
■我们确定了总共33项潜在研究。其中,最后在系统评价中考虑了10个,在荟萃分析中考虑了5个。总的来说,移植患者报告了HRQoL主要领域的良好评分。这些数据得到了meta分析的结果的证实,该结果显示了接受常规治疗的移植和β-TM患者在身体和情绪方面的显着差异。具有中等效应大小[d=0.65,95%CI(0.29-1.02),z=3.52,p=0.0004,I2=75%;d=0.59,95%CI(0.43-0.76),z=6.99,p<0.00001,I2=0%,分别]。
HRQoL在β-TM移植患者中通常是良好的,并且可以显著有助于决定是否移植用常规疗法治疗的β-TM患者。
UNASSIGNED: β-Thalassemia major (β-TM) represents one of the most important hemoglobinopathies worldwide. Remarkable improvements have been achieved in supportive therapy based on blood transfusions and iron chelation, and nowadays, this approach is capable of assuring a long life in these patients in industrialized countries. The only curative treatment is represented by hematopoietic stem cell transplantation (HSCT). However, this treatment may be burdened by deterioration in the Health-Related Quality of Life (HRQoL). This paper aimed to evaluate the role of HRQoL in transplanted β-TM patients with a systematic review and meta-analysis.
UNASSIGNED: PubMed database, Web of Science, and Scopus were systematically searched for studies published between January 1st, 2000 to September 2020. The following terms were entered in the database queries: β-thalassemia, HRQoL, and HSCT. The study was carried out according to the Preferred Reporting Items for Systematic and Meta-analyses (PRISMA) statement.
UNASSIGNED: We identified a total of 33 potential studies. Among these, 10 were finally considered in the systematic review and 5 in the meta-analysis. Overall, good scores in the principal domains of HRQoL were reported by transplanted patients. These data were confirmed by results of meta-analysis that showed significant difference between transplanted and β-TM patients treated with conventional therapy in the physical and emotional dimension, with a medium effect size [d=0.65, 95% CI (0.29-1.02), z = 3.52, p =0.0004, I2=75%; and d=0.59, 95% CI (0.43-0.76), z = 6.99, p <0.00001, I2=0%, respectively].
UNASSIGNED: HRQoL is generally good in β-TM transplanted patients and may significantly contribute in deciding whether or not to transplant a β-TM patient treated with conventional therapy.