Scientific Literature
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Biologic and Clinical Efficacy of LentiGlobin for Sickle Cell Disease
BACKGROUND Sickle cell disease is characterized by the painful recurrence of vaso-occlusive events. Gene therapy with the use of LentiGlobin for sickle cell disease (bb1111; lovotibeglogene autotemcel) consists of autologous…
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Iron Overload Status in Patients with Non-Transfusion-Dependent Thalassemia in China
Background: Non-transfusion-dependent thalassemia (NTDT) is a genetic disorder most commonly including beta-thalassemia intermedia (Beta-TI), HbE/Beta thalassemia (HbE/Beta thalassemia), and hemoglobin H disease (HbH disease). NTDT patients can be at risk of…
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Nutrition in Thalassemia: A Systematic Review of Deficiency, Relations to Morbidity, and Supplementation Recommendations
Reports of nutritional deficiencies in patients with thalassemia (Thal) are common. Despite its importance, however, nutritionally focused research in Thal has been limited by inadequate sample size, inconsistent methodology, a…
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Atrial Fibrillation in β-Thalassemia: Overview of Mechanism, Significance and Clinical Management
Atrial fibrillation in patients with β-thalassemia has a higher incidence compared to the general population. Its pathophysiology is peculiar and strictly related to anemia, iron overload, hemolysis, inflammation, atrial dilatation,…
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Guidelines for the Monitoring and Management of Iron Overload in Patients with Haemoglobinopathies and Rare Anaemias
These Guidelines were compiled according to the BSH process at https://b-s-h.org.uk/guidelines/proposing-and-writing-a-new-bsh-guideline/. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) nomenclaturewas used to evaluate levels of evidence and to assess…
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The Influence of Cardiovascular Risk Factors and Hypogonadism on Cardiac Outcomes in an Aging Population of Beta-Thalassemia Patients
Beta-thalassemia major (β-TM) is a hereditary genetic disease worsened by many comorbidities due to transfusion-related iron despite chelation therapy. Since there has recently been an increase in life expectancy of…
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Hepatocellular Carcinoma in Transfusion Dependent Thalassemia Patients: A Review from a Clinical Perspective
Survival in patients with transfusion-dependent thalassemias (TDT) has increased, and complications such as hepatocellular carcinoma (HCC) are emerging. Risk factors include viral infection, mainly hepatitis C virus (HCV), iron overload,…
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Evaluation of Stillbirth Among Pregnant People With Sickle Cell Trait
Importance: Relative to what is known about pregnancy complications and sickle cell disease (SCD), little is known about the risk of pregnancy complications among those with sickle cell trait (SCT).…
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Phylogenetic Analysis in the Clinical Risk Management of an Outbreak of Hepatitis C Virus Infection Among Transfused Thalassaemia Patients in Italy
Occurrence of hepatitis C virus (HCV) infection is reduced by effective risk management procedures, but patient-to-patient transmission continues to be reported in healthcare settings. To report the use of phylogenetic analysis in the…
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