Knowledge insufficient: the management of haemoglobin SC disease

Lydia H. Pecker, Beverly A. Schaefer, Lori Luchtman-Jones

Research output: Contribution to journalReview articlepeer-review

Abstract

Although haemoglobin SC (HbSC) accounts for 30% of sickle cell disease (SCD) in the United States and United Kingdom, evidence-based guidelines for genotype specific management are lacking. The unique pathology of HbSC disease is complex, characterized by erythrocyte dehydration, intracellular sickling and increased blood viscosity. The evaluation and treatment of patients with HbSC is largely inferred from studies of SCD consisting mostly of haemoglobin SS (HbSS) patients. These studies are underpowered to allow definitive conclusions about HbSC. We review the pathophysiology of HbSC disease, including known and potential differences between HbSS and HbSC, and highlight knowledge gaps in HbSC disease management. Clinical and translational research is needed to develop targeted treatments and to validate management recommendations for efficacy, safety and impact on quality of life for people with HbSC.

Original languageEnglish (US)
Pages (from-to)515-526
Number of pages12
JournalBritish journal of haematology
Volume176
Issue number4
DOIs
StatePublished - Feb 1 2017
Externally publishedYes

Keywords

  • haemoglobin SC
  • haemoglobin sickle C
  • sickle cell anaemia
  • sickle cell disease

ASJC Scopus subject areas

  • Hematology

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