title: Sickle Cell Disease: A Brief Update.
abstract: Sickle cell disease (SCD) is an inherited monogenic disease characterized by misshapen red blood cells that causes vaso-occlusive disease, vasculopathy, and systemic inflammation. Approximately 300,000 infants are born per year with SCD globally. Acute, chronic, and acute-on-chronic complications contribute to end-organ damage and adversely affect quantity and quality of life. Hematopoietic stem cell transplantation is the only cure available today, but is not feasible for the vast majority of people suffering from SCD. Fortunately, new therapies are in late clinical trials and more are in the pipeline, offering hope for this unfortunate disease, which has increasing global burden.
authors.affiliation: Division of Hematology and Medical Oncology, Department of Medicine, Oregon Health and Science University, 3181 Southwest Sam Jackson Park Road, Mailstop L586, Portland, OR 97239, USA. Electronic address: wong@ohsu.edu.
authors.forename: Sharl
authors.initials: S
authors.lastname: Azar
authors.affiliation: Division of Hematology/Oncology, Department of Pediatrics, Oregon Health and Science University, 3181 Southwest Sam Jackson Park Road, Mailstop CDRCP, Portland, OR 97239, USA; Division of Transfusion Services, Department of Pathology, Oregon Health and Science University, 3181 Southwest Sam Jackson Park Road, Mailstop HRC9, Portland, OR 97239, USA.
authors.forename: Trisha E
authors.initials: TE
authors.lastname: Wong
mesh_terms: D000755:Anemia, Sickle Cell / Q000235:genetics / Q000503:physiopathology* / Q000628:therapy*; D017707:Erythrocyte Transfusion; D018380:Hematopoietic Stem Cell Transplantation; D006451:Hemoglobin, Sickle / Q000235:genetics* / Q000378:metabolism; D006801:Humans; D006918:Hydroxyurea / Q000494:pharmacology / Q000627:therapeutic use; D007502:Iron Chelating Agents / Q000494:pharmacology / Q000627:therapeutic use; D011379:Prognosis; D012720:Severity of Illness Index
chemical_list: D006451:Hemoglobin, Sickle; D007502:Iron Chelating Agents; D006918:Hydroxyurea
keywords: Hemoglobinopathy; Hydroxyurea; Iron overload; Review; Sickle cell anemia; Sickle cell disease