Category: Sickle Cell News for February 2021

A systematic review of quality of life in sickle cell disease and thalassemia after stem cell transplant or gene therapy

Abstract Patients with sickle cell disease (SCD) and thalassemia experience several complications across their lifespan that lead to impairment in different health-related quality of life (HRQOL) domains. There is increasing interest in curative therapies for patients with SCD and thalassemia, including hematopoietic stem cell transplant (HSCT) and gene therapy; however, the effect of these therapies …

Influence of Nutrition on Disease Severity and Health-related Quality of Life in Adults with Sickle Cell Disease: A Prospective Study

Abstract Background & aims: Sickle cell disease (SCD), a genetic disorder resulting from the presence of a mutated hemoglobin S (HbS), has a worldwide distribution and causes significant morbidity and mortality in children and adults. Few studies addressed the determinants of SCD severity in adults; therefore, we investigated the impact of nutrition on the outcome of …

Transfusion and Cellular Therapy in Pediatric Sickle Cell Disease

Abstract Red blood cell (RBC) transfusion is critical in managing acute and chronic complications of sickle cell disease. Alloimmunization and iron overload remain significant complications of transfusion therapy and are minimized with prophylactic Rh and K antigen RBC matching and iron chelation. Matched sibling donor hematopoietic stem cell transplant (HSCT) is a curative therapeutic option. …