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- Title
Presence of the IVS-I-6-Mutated Allele in Beta-Thalassemia Major Patients Correlates with Extramedullary Hematopoiesis Incidence.
- Authors
Sousos, Nikolaos; adamidou, Despoina; Klonizakis, Philippos; agapidou, alexandra; Theodoridou, Stamatia; Spanos, Georgios; Psarras, Kyriakos; Vetsiou, Evaggelia; Vyzantiadis, Timoleon-achilleas; Vlachaki, Efthymia
- Abstract
Extramedullary hematopoiesis (EMH) results from the extension of hematopoietic tissue beyond the confines of the bones. Since the initiation of regular transfusion programs from an early age for all thalassemia major (TM) patients, EMH has not been considered a clinical issue anymore. The present study aims to record the prevalence of EMH in chronically transfused TM patients followed at our institution and to investigate possible risk factors associated with its occurrence. The project was designed as a retrospective, nonexperimental, descriptive, exploratory study. In total, the study enrolled 104 patients. EMH was revealed in 15/104 (14%) patients. The presence of intravening sequence (IVS)-I-6 was significantly related with the development of EMH (p < 0.05). No other demographic or biological factor studied was found to be related with the presence of EMH. The study stresses a profound incidence of asymptomatic EMH in a solid group of well-transfused TM patients. Given the high incidence of the IVS-I-6 allele in the Mediterranean and Middle Eastern region, high-quality, prospective, multicenter studies could confirm the association of EMH occurrence with the presence of the IVS-I-6 mutation and further evaluate the exact role of this mutation in the EMH process.
- Subjects
HEMATOPOIESIS; BETA-Thalassemia; PATIENT satisfaction; PATIENT compliance; PATIENTS; THERAPEUTICS
- Publication
Acta Haematologica, 2017, Vol 137, Issue 3, p175
- ISSN
0001-5792
- Publication type
Article
- DOI
10.1159/000463919