Evaluation of the Clinical and Laboratory Characteristics of Previously Followed-up Thalassemia Intermedia Patients to Provide Them Better Care in the Future. Issue 6 (August 2017)

• Record Type: Journal Article
• Authors: Tüfekçi, Özlem; Atabay, Berna; Türker, Meral; Yilmaz Bengoa, Şebnem; Gözmen, Salih; Karapinar, Tuba; Ören, Hale; İrken, Gülersu
• Is Part Of: Journal of pediatric hematology/oncology. Volume 39:Issue 6(2017)
• Publication Date: 2017-08
• Subjects: thalassemia intermedia -- transfusion -- chelation -- morbidity; Pediatric hematology -- Periodicals; Tumors in children -- Periodicals; 618.9215
• DOI: 10.1097/MPH.0000000000000914
• Languages: English
• ISSNs: 1077-4114

Abstract

Abstract : The increased awareness about the severity of complications in thalassemia intermedia patients led authorities to develop strategies for better management and follow-up of these patients. In this study, we aimed to define the clinical and laboratory characteristics in previously followed-up β-thalassemia intermedia patients and wanted to gain an insight about the follow-up of this patient population in a developing country to provide them better care in the future. The mean age at diagnosis was 4 years, and the mean hemoglobin was 7.13 g/dL. The mean age at the beginning of regular transfusion was 4.8 years. An overall 74% of patients were on a regular transfusion program. The mean ferritin values at diagnosis and the last follow-up were 487 and 1225 ng/mL, respectively. The most common mutations detected in patients were IVS-I-110, IVS-I-6, IVS-II-1, and FCS 8/9 in order of frequency. Complications were seen in 48% of patients. The most common complications were osteopenia/osteoporosis (34%), growth retardation (24%), hypogonadism (18%), and cardiomyopathy (13%). In conclusion, the relatively higher complication rate in our patients who were previously treated highlights once again the need for an increased effort for optimal management and follow-up of this specific group of patients.