Mechanisms of Renal Disease in β-Thalassemia

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Aspirin Resistance

The development of adverse cardiovascular events despite aspirin use has established an interest in a possible resistance to the drug. Several definitions have been set and various laboratory testing modalities are available. This has led to a wide range of prevalence reports in different clinical entities. The etiologic mechanism has been related to clinical, genetic, and other miscellaneous factors. The clinical implications of this phenomenon are significant and warrant concern. Management strategies are currently limited to dosing alteration and introduction of other anitplatelet agents. However, these measures have not met the expected efficacy or safety

Thalassemia and Venous Thromboembolism

Although the life expectancy of thalassemia patients has markedly improved over the last few decades, patients still suffer from many complications of this congenital disease. The presence of a high incidence of thromboembolic events, mainly in thalassemia intermedia, has led to the identification of a hypercoagulable state in these patients. In this review, the molecular and cellular mechanisms leading to hypercoagulability in thalassemia are highlighted, with a special focus on thalassemia intermedia being the group with the highest incidence of thrombotic events as compared to other types of thalassemia. Clinical experience and available clues on optimal management are also discussed

Burkitt's lymphoma of the colon and bronchi: three case reports

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Thalassaemia Intermedia: an Update

Our understanding of the molecular and pathophysiological mechanisms underlying the disease process in patients with thalassaemia intermedia (TI) has substantially increased over the past decade. TI encompasses a wide clinical spectrum of beta-thalassaemia phenotypes. Some TI patients are asymptomatic until adult life, whereas others are symptomatic from as young as 2 years of age. A number of clinical complications commonly associated with TI are rarely seen in thalassaemia major, including extramedullary hematopoiesis, leg ulcers, gallstones, thrombosis and pulmonary hypertension. There are a number of options currently available for managing patients with TI, including transfusion therapy, iron chelation therapy, modulation of foetal haemoglobin production and haematopoietic stem cell transplantation. However, at present, there are no clear guidelines for an orchestrated optimal treatment plan

Coagulopathy in Beta-Thalassemia: Current Understanding and Future Perspectives

As the life expectancy of β-thalassemia patients has markedly improved over the last decade, several new complications are being recognized. The presence of a high incidence of thromboembolic events, mainly in thalassemia intermedia patients, has led to the identification of a hypercoagulable state in thalassemia. In this review, the molecular and cellular mechanisms leading to hypercoagulability in thalassemia are highlighted, and the current clinical experience is summarized. Recommendations for thrombosis prophylaxis are also discussed

2021 update on clinical trials in β‐thalassemia

AbstractThe treatment landscape for patients with β‐thalassemia is witnessing a swift evolution, yet several unmet needs continue to persist. Patients with transfusion‐dependent β‐thalassemia (TDT) primarily rely on regular transfusion and iron chelation therapy, which can be associated with considerable treatment burden and cost. Patients with non‐transfusion‐dependent β‐thalassemia (NTDT) are also at risk of significant morbidity due to the underlying anemia and iron overload, but treatment options in this patient subgroup are limited. In this review, we provide updates on clinical trials of novel therapies targeting the underlying pathology in β‐thalassemia, including the α/non‐α‐globin chain imbalance, ineffective erythropoiesis, and iron dysregulation

Antithrombotic Prophylaxis in the Middle East

Several factors have been proposed to explain the persistence of a high incidence of venous thromboembolism worldwide with its associated morbidity and mortality. Underutilization of anticoagulants and failure of adherence to thromboprophylaxis guidelines are emerging global health concerns. We herein review this alarming observation with special emphasis on the Middle East region. We also discuss strategies that could help control this increasingly reported problem

‘Phenoconversion’ in adult patients with β-thalassemia

To the Editor: Patients with clinically significant forms of β-thalassemia have been historically classified as having a β-thalassemia major or β-thalassemia intermedia phenotype, with the first primarily referring to patients who present with severe anemia during early childhood and require lifelong transfusion therapy and the latter relating to patients who present later in childhood or adolescence with mild–moderate anemia that does not necessitate immediate commitment to regular transfusion therapy.1 In more recent years, the terms transfusion-dependent β-thalassemia (TDT) and non-transfusion-dependent β-thalassemia (NTDT) gradually replaced the two conventional phenotypes, respectively, to highlight the importance of transfusion-dependence in determining the dominant pathophysiology and treatment needs.2 International management guidelines and clinical trial eligibility criteria are now clearly differentiated for TDT and NTDT.3,4 Based on clinical observation, NTDT patients may still require occasional transfusion therapy in cases of infection, pregnancy, or surgery, while some go on to receive more frequent transfusions or become transfusiondependent for various clinical reasons or as heir disease progresses. The rate and determinants of such ‘phenoconversion’ from NTDT to TDT remain poorly understood and have never been previously evaluated. Such data remain essential to fully understand the natural course of β-thalassemia as a disease spectrum