An overview on thalassemia and challenges during COVID-19

Abstract

The thalassemia is a group of disorders in which the normal ratio of alpha globin to beta globin production is disrupted due to a disease-causing variant in one or more of the globin genes. This abnormal alpha- to beta-chain ratio causes the unpaired chains to precipitate and causes destruction of red blood cell precursors in the bone marrow (ineffective erythropoiesis) and circulation (hemolysis). Affected individuals with thalassemia have variable degrees of anemia and extramedullary hematopoiesis, which in turn can cause bone changes, impaired growth, and iron overload. The aim and objectives are to describe the basic genetic differences between alpha-thalassemia and beta-thalassemia. Describe the hematologic findings and pathophysiological changes that are associated with beta-thalassemia major. Summarize the etiology of thalassemias and describe the basic genetic differences between alpha-thalassemia and beta-thalassemia. Describe the genetic, hematologic, and clinical differences between alpha-thalassemia trait, hemoglobin H disease, and hydrops fetalis. outline the challenges of thalassemia during Covid 19 crisis. Thalassemia is the most common, inherited, single-gene disorder in the world. Treatment of the inherited blood disorder thalassemia depends upon the level of severity.

Thalassemia and pregnancy complications

“…The excess γ chains form tetramers which are poor carriers of O2 since their affinity for O2 is too high, so it is not dissociated in the periphery. Homozygote α0 thalassemias, where numerous γ4 but no α-globin's occur at all (referred to as Hb Barts), often result in death soon after birth [14,15,16,17].…”

“…Various treatment options, such as blood transfusion or postpartum prophylaxis for thromboembolism may be indicated. However, since prevention is always better than cure, antenatal screening and an accurate genetic prenatal diagnosis should be preferably achieved during early gestation [17,22,23]. Also, Sutcu et al, reported a patient having repeated pregnancies with nonimmune hydrops fetalis caused by alpha thalassemia and concluded that the parents who are alpha-thalassemia carriers have 25% chance of having of a child with major alpha-thalassemia.…”

Role of feature tracking cardiac magnetic resonance imaging in early detection of cardiac dysfunction in β‐thalassemia patients recovered from COVID‐19: A cross‐sectional study

“…13 Patients with chronic diseases such as thalassemia are at greater risk of developing COVID-19 complications. 14 β-Thalassemia is the world's most common form of thalassemia. 15 It is characterized with chronic high burden of inflammation.…”

Comparing serum ferritin levels during COVID-19 infection and recovery period in pediatric patients with transfusion-dependent thalassemia, a single-center study

“…β-thalassemia is divided into two groups: transfusion-dependent β-thalassemia (TDT) and non-transfusion-dependent β-thalassemia (NTDT) ( 6 , 9 ). TDT children might be at risk for severe disease of COVID-19 because of several complications like iron overload, ineffective erythropoiesis, hypercoagulability, splenectomy, and multiple organ damage due to excess iron ( 6 , 10 ). These complications may increase the susceptibility to infection in TDT children ( 11 ).…”