Non-Transfusion-Dependent Thalassemia: An Update on Complications and Management

dc.contributor.authorSleiman, Joseph
dc.contributor.authorTarhini, Ali
dc.contributor.authorBou-Fakhredin, Rayan
dc.contributor.authorSaliba, Antoine N.
dc.contributor.authorCappellini, Maria Domenica
dc.contributor.authorTaher, Ali T.
dc.contributor.departmentMedicine, School of Medicineen_US
dc.date.accessioned2018-07-24T13:56:50Z
dc.date.available2018-07-24T13:56:50Z
dc.date.issued2018-01-08
dc.description.abstractPatients with non-transfusion-dependent thalassemia (NTDT) experience many clinical complications despite their independence from frequent transfusions. Morbidities in NTDT stem from the interaction of multiple pathophysiological factors: ineffective erythropoiesis, iron overload (IOL), and hypercoagulability. Ineffective erythropoiesis and hemolysis are associated with chronic hypoxia and a hypercoagulable state. The latter are linked to a high prevalence of thromboembolic and cerebrovascular events, as well as leg ulcers and pulmonary hypertension. IOL in NTDT patients is a cumulative process that can lead to several iron-related morbidities in the liver (liver fibrosis), kidneys, endocrine glands (endocrinopathies), and vascular system (vascular disease). This review sheds light on the pathophysiology underlying morbidities associated with NTDT and summarizes the mainstays of treatment and some of the possible future therapeutic interventions.en_US
dc.eprint.versionFinal published versionen_US
dc.identifier.citationSleiman, J., Tarhini, A., Bou-Fakhredin, R., Saliba, A. N., Cappellini, M. D., & Taher, A. T. (2018). Non-Transfusion-Dependent Thalassemia: An Update on Complications and Management. International Journal of Molecular Sciences, 19(1), 182. http://doi.org/10.3390/ijms19010182en_US
dc.identifier.urihttps://hdl.handle.net/1805/16774
dc.language.isoen_USen_US
dc.publisherMDPIen_US
dc.relation.isversionof10.3390/ijms19010182en_US
dc.relation.journalInternational Journal of Molecular Sciencesen_US
dc.rightsAttribution 3.0 United States
dc.rights.urihttps://creativecommons.org/licenses/by/3.0/us
dc.sourcePMCen_US
dc.subjectIneffective erythropoiesisen_US
dc.subjectIron chelation therapyen_US
dc.subjectIron overloaden_US
dc.subjectManagementen_US
dc.subjectMorbidityen_US
dc.subjectNon-transfusion dependent thalassemiaen_US
dc.titleNon-Transfusion-Dependent Thalassemia: An Update on Complications and Managementen_US
dc.typeArticleen_US
Files
Original bundle
Now showing 1 - 1 of 1
Loading...
Thumbnail Image
Name:
ijms-19-00182.pdf
Size:
781.89 KB
Format:
Adobe Portable Document Format
Description:
Main article
License bundle
Now showing 1 - 1 of 1
No Thumbnail Available
Name:
license.txt
Size:
1.99 KB
Format:
Item-specific license agreed upon to submission
Description: