Thalassaemia

AT Taher, DJ Weatherall, MD Cappellini - The Lancet, 2018 - thelancet.com
Inherited haemoglobin disorders, including thalassaemia and sickle-cell disease, are the
most common monogenic diseases worldwide. Several clinical forms of α-thalassaemia and …

How I manage medical complications of β-thalassemia in adults

AT Taher, MD Cappellini - Blood, the Journal of the American …, 2018 - ashpublications.org
The complex pathophysiology in β-thalassemia can translate to multiple morbidities that
affect every organ system. Improved survival due to advances in management means that …

Cardiovascular function and treatment in β-thalassemia major: a consensus statement from the American Heart Association

DJ Pennell, JE Udelson, AE Arai, B Bozkurt… - Circulation, 2013 - Am Heart Assoc
This aim of this statement is to report an expert consensus on the diagnosis and treatment of
cardiac dysfunction in β-thalassemia major (TM). This consensus statement does not cover …

Deferasirox, deferiprone and desferrioxamine treatment in thalassemia major patients: cardiac iron and function comparison determined by quantitative magnetic …

A Pepe, A Meloni, M Capra, P Cianciulli… - …, 2010 - pmc.ncbi.nlm.nih.gov
Background Oral deferiprone was suggested to be more effective than subcutaneous
desferrioxamine for removing heart iron. Oral once-daily chelator deferasirox has recently …

The effect of deferasirox on cardiac iron in thalassemia major: impact of total body iron stores

JC Wood, BP Kang, A Thompson… - Blood, The Journal …, 2010 - ashpublications.org
We present results from a prospective, multicenter, open-label, single-arm study evaluating
response of cardiac and liver iron to deferasirox therapy for 18 months. Twenty-eight …

Health-related quality of life (HRQoL) in beta-thalassemia major (β-TM) patients assessed by 36-item short form health survey (SF-36): a meta-analysis

M Arian, M Mirmohammadkhani, R Ghorbani… - Quality of Life …, 2019 - Springer
Purpose The main purpose of this meta-analysis was to evaluate the impact of beta-
thalassemia major (BTM) on the health-related quality of life as assessed by the medical …

Deferasirox for managing iron overload in people with thalassaemia

C Bollig, LK Schell, G Rücker, R Allert… - Cochrane database …, 2017 - cochranelibrary.com
Background Thalassaemia is a hereditary anaemia due to ineffective erythropoiesis. In
particular, people with thalassaemia major develop secondary iron overload resulting from …

Effects of deferasirox-deferoxamine on myocardial and liver iron in patients with severe transfusional iron overload

Y Aydinok, A Kattamis, MD Cappellini… - Blood, The Journal …, 2015 - ashpublications.org
Deferasirox (DFX) monotherapy is effective for reducing myocardial and liver iron
concentrations (LIC), although some patients may require intensive chelation for a limited …

Oral chelators in transfusion-dependent thalassemia major patients may prevent or reverse iron overload complications

K Farmaki, I Tzoumari, C Pappa - Blood Cells, Molecules, and Diseases, 2011 - Elsevier
Combined chelation treatment may be a better approach for transfusion-dependent
thalassemia major patients with iron overload complications because of increased efficacy …

[HTML][HTML] International survey of T2* cardiovascular magnetic resonance in β-thalassemia major

JP Carpenter, M Roughton, DJ Pennell… - …, 2013 - ncbi.nlm.nih.gov
Accumulation of myocardial iron is the cause of heart failure and early death in most
transfused thalassemia major patients. T2* cardiovascular magnetic resonance provides …