Sickle cell disease: time for a closer look at treatment options? Review uri icon

Overview

MeSH Major

  • Anemia, Sickle Cell
  • Hematopoietic Stem Cell Transplantation
  • Hydroxyurea

abstract

  • Tremendous progress has been made in the care of individuals with sickle cell over the past several decades. Major successes have been comprehensive infection prophylaxis, prediction and prevention of stroke, and better transfusion care, the latter including both prevention of alloimmunization and treatment of iron overload. However, definitive therapies remain limited to hydroxycarbamide (hydroxyurea) and stem cell transplantation, both of which have been in use for at least two decades. Despite knowing the progressive natural history of the disease with organ dysfunction, failure, and ultimately death at a young age, definitive therapies are considered for only a small proportion of individuals. Consequently, while life expectancy has improved dramatically from the last century, the ongoing pace of advancement has slowed or stalled. We believe that it is time to broaden the use of definitive therapy for those with asymptomatic disease, being cautiously more aggressive in our approach.

publication date

  • August 2013

Research

keywords

  • Review

Identity

Language

  • eng

Digital Object Identifier (DOI)

  • 10.1111/bjh.12413

PubMed ID

  • 23772687

Additional Document Info

start page

  • 455

end page

  • 64

volume

  • 162

number

  • 4