Google
Trusted Resources: Evidence & Education
Scientific literature and patient education texts
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
read more
+myBinderRelated Content
-
Today’s Faces of Sickle Cell Disease: Rodrick MurrayRodrick Murray was 3 months old when he ...
-
State of Sickle Cell DiseaseOver the past century, great advances ha...
-
Inhaled steroids reduce pain and sVCAM levels in individuals with sickle cell disease: A triple-blind, randomized tr...Clinical and preclinical data demonstrat...
-
Malaria-Driven Sickle Cell Trait Selection – Evidence for Modern Day Human Evolution?Sickle cell disease poses a serious heal...
-
Meet the oneSCDvoice community!https://www.onescdvoice.com/wp-content/u...
-
Review Board Finds Global Blood’s Sickle Cell Disease Therapy Voxelotor Is SafeAn independent review board has found Gl...
-
Kids with sickle cell disease aren’t receiving key vaccines, Michigan study findsA significant proportion of children w...
To improve your experience on this site, we use cookies. This includes cookies essential for the basic functioning of our website, cookies for analytics purposes, and cookies enabling us to personalize site content. By clicking on 'Accept' or any content on this site, you agree that cookies can be placed. You may adjust your browser's cookie settings to suit your preferences. More Information
The cookie settings on this website are set to "allow cookies" to give you the best browsing experience possible. If you continue to use this website without changing your cookie settings or you click "Accept" below then you are consenting to this.
Powered by
Support for this site is provided by
This platform is made possible through a partnership with the Sickle Cell Disease Association of America, Inc. (SCDAA) and its member organizations. SCDAA's mission is to advocate for people affected by sickle cell conditions and empower community-based organizations to maximize quality of life and raise public consciousness while advancing the search for a universal cure.