Purpose: With regular blood transfusions and modern medical management strategies, transfusion-dependent thalassemia (TDT) patients are currently living into adulthood, but they still suffer from the chronicity of the disease and its complications. This study was aimed to assess the health-related quality of life (HRQoL) scores in pediatric TDT patients compared with healthy controls.
Patients And Methods: The PedsQL 4.0 Generic Core Scales were administered to 80 pediatric TDT patients (aged 5 to 18 y, child self-report) and their parents (parent-proxy report), as well as age-matched and sex-matched healthy controls (80 participants and their parents).
Results: Patients with TDT and their parents rated lower HRQoL scores in all domains (physical, emotional, social, and school functioning) compared with the healthy population (P<0.01). The univariate analysis indicated that the total HRQoL score for children's self-reports was negatively predicted by high ferritin levels and the presence of any complication. The only negative predictor of the total HRQoL score in multivariate analyses was a high ferritin level (>1800 ng/dL).
Conclusions: This study showed that thalassemia has a negative impact on HRQoL when compared with a healthy population. For a better quality of life, intensive treatment should be applied for complications and high ferritin levels.
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http://dx.doi.org/10.1097/MPH.0000000000000790 | DOI Listing |
Mymensingh Med J
January 2025
Dr Khaza Amirul Islam, Medical Officer (Haematology), Shaheed Ziaur Rahman Medical College Hospital, Bogra, Bangladesh; E-mail:
Transfusion dependent thalassemia (TDT) patients require a regular blood transfusion to survive. Without adequate transfusion support, they suffer many complications, and have a short life span. Near about 200 milligrams of iron remains within a single Red cell concentrate (RCC) unit.
View Article and Find Full Text PDFPrimary gastric T-cell lymphomas (PGTL) are exceedingly rare with an estimated incidence of 0.0091 per 100,000 person-years, affecting mainly elderly males. PGTL can present with a variety of gastrointestinal symptoms, but patients only rarely present with perforation.
View Article and Find Full Text PDFCase Rep Hematol
December 2024
Department of Pathology and Laboratory Medicine, University of California Irvine (UCI) Medical Center, Orange, USA.
Chronic myelomonocytic leukemia (CMML) is a myelodysplastic/myeloproliferative neoplasm characterized by peripheral blood monocytosis and bone marrow dysplasia. In approximately one-fourth of cases, CMML can demonstrate progression to acute myeloid leukemia (AML), referred to as AML ex CMML. We present a 58-year-old woman with a past medical history of idiopathic thrombocytopenic purpura (ITP) who demonstrated 24% bone marrow blasts on a repeat biopsy obtained two years after being diagnosed with CMML.
View Article and Find Full Text PDFCureus
November 2024
Department of Neurosurgery, Tokyo Women's Medical University, Tokyo, JPN.
Introduction: The ZAP-X® Gyroscopic Radiosurgery System (ZAP Surgical Systems, Inc., San Carlos, CA, USA) is expected to be a highly accurate next-generation treatment system that enables gyro-stereotactic irradiation of intracranial lesions. In this study, we report the initial treatment course using ZAP-X for intracranial lesions that recurred after Gamma Knife (GK) treatment.
View Article and Find Full Text PDFStem Cell Res Ther
December 2024
Department of Pediatrics, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, No.107, West Yan Jiang Road, Guangzhou, 510120, Guangdong, China.
Background: Allo-HSCT is a curative therapy for patients with transfusion-dependent thalassemia (TDT). The high incidence of transplant-related complications is becoming an obstacle to safe and effective unrelated donor (URD) transplantation.
Methods: In this retrospective study, we reported the survival outcomes and complications of transplantation in thalassemia patients using a novel regimen consisting of pre-transplantation immunosuppression (PTIS) and modified myeloablative conditioning based on intravenous busulfan, cyclophosphamide, fludarabine, and rabbit anti-human thymocyte immunoglobulin.
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