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Sickle cell disease: Challenges and progress
source: Blood
year: 2016
authors: Leslie V. Parise, Nancy Berliner
summary/abstract:Despite a long history of knowing the genetic cause of sickle cell disease (SCD), progress in developing treatments to prevent painful vaso-occlusive crises and the other myriad of associated symptoms has, until recently, been disappointingly slow. As long ago as 1949, Pauling et al described sickle cell anemia as a molecular disease, with two other groups convincingly describing it as an inherited disorder. Details of the mutation (replacement of glutamic acid with valine in the 6th position of the hemoglobin [Hb] β-chain) were first described by Ingram in 1956. Despite these early discoveries, the life expectancy of sickle cell patients only began to improve significantly within the last 30 years, first with the introduction of prophylactic penicillin V in the 1980s, followed by more aggressive blood transfusions, and in 1998, with the introduction of hydroxyurea as a mainstay of treatment.
Beyond the mutation of Hb as the cause of SCD, Hebbel et al initiated a new era of research in 1980 by introducing the concept that sickle red blood cells (RBCs) are abnormally adhesive. Many subsequent studies from Hebbel et al and others led to the realization that not only sickle RBCs but other blood cells, especially leukocytes and platelets, are activated and have the potential to contribute to vaso-occlusive crises. This is a backdrop for the current review series. Over the last few years, our understanding of the complexities of cellular, plasma, and genetic contributors to the various symptoms of SCD has accelerated. New drugs and genetic cures are on the horizon. In this review series, five leading groups provide updates on important aspects of SCD.
organization: BloodDOI: 10.1182/blood-2015-12-674606
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