Care of patients with hemoglobin disorders during the COVID-19 pandemic: An overview of recommendations.

The outbreak of Coronavirus Disease 2019 (COVID-19) caused by the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has led to a global health emergency.1 Compared to the general population, patients with hemoglobin disorders such as sickle cell disease (SCD) or thalassemia are expected to be more severely affected by COVID-19 due to their preexisting chronic morbidities.2 The Centers for Disease Control and Prevention does not report any specific indications for patients with hemoglobinopathies. However, it can be hypothesized that the rapid spread of the virus may render these patients fragile when fighting the infection. SCD, a hematological condition with functional asplenia, puts patients at a greater risk to develop acute pulmonary complications, including viral infections.2 A study by Hussain et al reported four SCD cases that tested positive for COVID-19.3 These cases initially presented to the emergency department for a typical vaso-occlusive crisis (VOC), and the clinical course of their SARS-CoV-2 infection was rather mild. Patients had a history of respiratory complications, such as acute chest syndrome (ACS), asthma, or pulmonary embolism, which may be potential risk factors for progressive COVID-19 pulmonary disease in patients with SCD.3 A series of isolated cases of ACS in SCD patients positive for COVID-19 has been recently reported.4,5 Therefore, very little clinical experience of infected patients with SCD currently exists. For this reason, we believe that certain recommendations must be followed by healthcare professionals treating any SCD patient infected with SARS-CoV-2

Iron Overload and Chelation Therapy in Non-Transfusion Dependent Thalassemia

Iron overload (IOL) due to increased intestinal iron absorption constitutes a major clinical problem in patients with non-transfusion-dependent thalassemia (NTDT), which is a cumulative process with advancing age. Current models for iron metabolism in patients with NTDT suggest that suppression of serum hepcidin leads to an increase in iron absorption and subsequent release of iron from the reticuloendothelial system, leading to depletion of macrophage iron, relatively low levels of serum ferritin, and liver iron loading. The consequences of IOL in patients with NTDT are multiple and multifactorial. Accurate and reliable methods of diagnosis and monitoring of body iron levels are essential, and the method of choice for measuring iron accumulation will depend on the patient's needs and on the available facilities. Iron chelation therapy (ICT) remains the backbone of NTDT management and is one of the most effective and practical ways of decreasing morbidity and mortality. The aim of this review is to describe the mechanism of IOL in NTDT, and the clinical complications that can develop as a result, in addition to the current and future therapeutic options available for the management of IOL in NTDT

Beta Thalassemia: New Therapeutic Options Beyond Transfusion and Iron Chelation

Hemoglobinopathies are among the most common monogenic diseases worldwide. Approximately 1-5% of the global population are carriers for a genetic thalassemia mutation. The thalassemias are characterized by autosomal recessive inherited defects in the production of hemoglobin. They are highly prevalent in the Mediterranean, Middle East, Indian subcontinent, and East and Southeast Asia. Due to recent migrations, however, the thalassemias are now becoming more common in Europe and North America, making this disease a global health concern. Currently available conventional therapies in thalassemia have many challenges and limitations. A better understanding of the pathophysiology of \u3b2-thalassemia in addition to key developments in optimizing transfusion programs and iron-chelation therapy has led to an increase in the life span of thalassemia patients and paved the way for new therapeutic strategies. These can be classified into three categories based on their efforts to address different features of the underlying pathophysiology of \u3b2-thalassemia: correction of the globin chain imbalance, addressing ineffective erythropoiesis, and improving iron overload. In this review, we provide an overview of the novel therapeutic approaches that are currently in development for \u3b2-thalassemia

Recommendations for pregnancy in Fanconi anemia

Introduction: Fanconi anemia (FA) is a rare congenital disease that belongs to the family of congenital trilinear bone marrow failure. Most FA patients will suffer bone marrow failure and the main treatment relies on supportive measures or more recently on the use of hematopoietic stem cell transplant. The improvements seen in the management of FA has led women to reach childbearing age and have successful pregnancies. However, these pregnancies are associated with increased complications such as preterm delivery, cesarean delivery, eclampsia and others. Areas covered: This review highlights on the outcome of pregnancies in FA patients reported in the literature along with practical recommendations. Expert opinion: Multidisciplinary efforts are required to optimize the management of pregnancy in FA patients. Moreover, the development of a set of recommendations to optimize the treatment is highly necessary