Quality Of Life In Patients With Thalassemia Major After Hematopoietic Stem-Cell Transplantation

Objective:To investigate and assess the health-related quality of life (HRQoL) of patients who underwent allogeneic HSCT with thalassemia major and explore the influencing factors of the quality of life order to establish scientific basis to improve their quality of life.Methods:We use child-self and parent-proxy reports of Pediatric Quality of Life Inventory Measurement Models 4.0 (PedsQL4.0) to investigate and assess the HRQoL in 82 patients who underwent HLA-matched allogeneic HSCT in our hospital with thalassemia major from April 2005 to march 2014. Then to compare transplanted patients’ quality of life with nontransplanted thalassemia major patients and normal children with matchable age and sex.The influencing factors of HRQoL were analyzed with multivariate linear regression.Results:The quality of life scores of patients who underwent HLA-matched allogeneic HSCT with thalassemia major are higher than non-transplanted thalassemia major patients’in the physical health, emotional functioning, social functioning and school functioning domains,which is significantly different(P<0.05), especially in physical health domain and school functioning; The quality of life scores of patients who underwent HLA-matched allogeneic HSCT with thalassemia major are similar with normal children in physical health, role emotional and social function domains (P> 0.05).Multivariate linear regression analyses indicate that the time interval between hematopoietic stem cell transplantion and evaluation, level of serum ferritin when the evaluation took place,cGVHD and dependence on immunosuppressant and are associated with HRQoL significantly (P<0.05).Conclusions:The quality of life scores of patients who underwent HLA-matched allogeneic HSCT with thalassemia major are higher than nontransplanted thalassemia major patients’and the the quality of life scores similar with normal persons; The quality of life of patients who underwent allogeneic HSCT with thalassemia major is correlated with time interval between hematopoietic stem cell transplantion and evaluation, level of serum ferritin when the evaluation took place,cGVHD and dependence on immunosuppressant. We should strengthen the prevention of disease-related complication and the management of normative iron chelation therapy to improve their quality of life.