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 …

β-Thalassemia

R Origa - Genetics in Medicine, 2017 - nature.com
Abstract β-Thalassemia is caused by reduced (β+) or absent (β 0) synthesis of the β-globin
chains of hemoglobin. Three clinical and hematological conditions of increasing severity are …

Clinical classification, screening and diagnosis for thalassemia

V Viprakasit, S Ekwattanakit - Hematology/Oncology Clinics, 2018 - hemonc.theclinics.com
Over the past decade, our knowledge of the clinical diagnosis and management of
thalassemia has progressed extensively. In recent years, the most critical change in clinical …

[HTML][HTML] Non-transfusion-dependent thalassemias

KM Musallam, S Rivella, E Vichinsky… - …, 2013 - ncbi.nlm.nih.gov
Non-transfusion-dependent thalassemias include a variety of phenotypes that, unlike
patients with beta (β)-thalassemia major, do not require regular transfusion therapy for …

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 …

Optimal management of β thalassaemia intermedia

AT Taher, KM Musallam, MD Cappellini… - British journal of …, 2011 - Wiley Online Library
Our understanding of the molecular and pathophysiological mechanisms underlying the
disease process in patients with β thalassaemia intermedia (TI) has substantially increased …

[HTML][HTML] Elevated liver iron concentration is a marker of increased morbidity in patients with β thalassemia intermedia

KM Musallam, MD Cappellini, JC Wood, I Motta… - …, 2011 - ncbi.nlm.nih.gov
Background Patients with β thalassemia intermedia can have substantial iron overload,
irrespectively of their transfusion status, secondary to increased intestinal iron absorption …

[HTML][HTML] Management of transfusion-dependent β-thalassemia (TDT): Expert insights and practical overview from the Middle East

A El-Beshlawy, H Dewedar, S Hindawi, S Alkindi… - Blood Reviews, 2024 - Elsevier
Abstract β-Thalassemia is one of the most common monogenetic diseases worldwide, with a
particularly high prevalence in the Middle East region. As such, we have developed long …

β-thalassemia intermedia: a clinical perspective

KM Musallam, AT Taher… - Cold Spring …, 2012 - perspectivesinmedicine.cshlp.org
Our understanding of the molecular and pathophysiological mechanisms underlying the
disease process in patients with β-thalassemia intermedia has substantially increased over …

Short‐and long‐term risks of splenectomy for benign haematological disorders: should we revisit the indications?

F Rodeghiero, M Ruggeri - British journal of haematology, 2012 - Wiley Online Library
Splenectomy has represented a key treatment option in the treatment of many benign
haematological diseases, including immune thrombocytopenia (ITP) and disorders …