Minireview: Clinical severity in sickle cell disease: the challenges of definition and prognostication

Exp Biol Med (Maywood). 2016 Apr;241(7):679-88. doi: 10.1177/1535370216640385. Epub 2016 Mar 23.


Sickle cell disease (SCD) is a monogenic, yet highly phenotypically variable disease with multisystem pathology. This manuscript provides an overview of many of the known determinants, modifiers, and correlates of disease severity in SCD. Despite this wealth of data, modeling the variable and multisystem pathology of SCD continues to be difficult. The current status of prediction of specific adverse outcomes and global disease severity in SCD is also reviewed, highlighting recent successes and ongoing challenges.

Keywords: Biomarkers; disease severity; models; outcomes; phenotype; prediction; prevention; prognosis; sickle cell anemia; sickle cell disease; survival.

Publication types

  • Review

MeSH terms

  • Anemia, Sickle Cell / blood
  • Anemia, Sickle Cell / diagnosis*
  • Anemia, Sickle Cell / genetics
  • Anemia, Sickle Cell / pathology
  • Fetal Hemoglobin / analysis
  • Humans
  • Prognosis
  • Severity of Illness Index
  • beta-Globins / analysis


  • beta-Globins
  • Fetal Hemoglobin