Sickle cell disease
Neha Bhasin
Department of Pediatrics, Division of Hematology, UCSF Benioff Children's Hospital Oakland, Oakland, CA, USA
Search for more papers by this authorElliott P Vichinsky
Department of Pediatrics, Division of Hematology, UCSF Benioff Children's Hospital Oakland, Oakland, CA, USA
Search for more papers by this authorNeha Bhasin
Department of Pediatrics, Division of Hematology, UCSF Benioff Children's Hospital Oakland, Oakland, CA, USA
Search for more papers by this authorElliott P Vichinsky
Department of Pediatrics, Division of Hematology, UCSF Benioff Children's Hospital Oakland, Oakland, CA, USA
Search for more papers by this authorAdam J Mead PhD, FRCP, FRCPath, FMedSci
Haematopoietic Stem Cell Biology Laboratory, Medical Research Council Molecular Haematology Unit, Medical Research Council Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, UK
Search for more papers by this authorMichael A Laffan DM, MRCP, FRCPath
Department of Immunology and Inflammation, Faculty of Medicine, Imperial College London, London, UK
Search for more papers by this authorGraham P Collins DPhil, FRCP, FRCPath
Department of Haematology, Oxford Cancer and Haematology Centre, Oxford, UK
Search for more papers by this authorDeborah Hay DPhil, MRCP, FRCPath
Nuffield Division of Clinical Laboratory Sciences, Radcliffe Department of Medicine, University of Oxford, Oxford, UK
Search for more papers by this authorA Victor Hoffbrand MA, DM, FRCP, FRCPath, FRCP (Edin), DSc, FMedSci
Emeritus Professor of Haematology Honorary Consultant Haematologist
University College London, London, UK
Royal Free Hospital, London, UK
Search for more papers by this authorSummary
Sickle cell disease (SCD) is an inherited red blood cell disorder that causes chronic haemolytic anaemia and vasculopathy with multiple clinical complications. Deoxygenation of haemoglobin S leads to a conformational change in the red cell structure leading to these complications. Here we discuss the demographic distribution of the disease, genotypes, pathophysiology, diagnostic modalities, clinical manifestations and therapies available to date for SCD.
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