Considering the spleen in sickle cell disease

Sara El Hoss, Valentine Brousse

Research output: Contribution to journalReview articlepeer-review

25 Citations (Scopus)

Abstract

Introduction: In human physiology, the spleen is generally neglected, and its role is considered anecdotal. In sickle cell disease, splenic dysfunction is the main cause of life-threatening complications, particularly in early childhood with the risk of pneumococcal overwhelming sepsis and acute splenic sequestration crisis, notably. During the course of the disease, the spleen functionally declines and anatomically disappears, albeit with great individual variability depending on modulating genetic and environmental factors. Areas covered: The present review aims to provide an overview of spleen structure and function in order to highlight its role in sickling disorders. The clinical features of spleen damage in sickle cell disease, as well as complications and short- and long-term consequences, are reviewed, along with the main therapeutic options. Expert opinion: Management of acute splenic sequestration recurrence and timing of splenectomy in children with sickling disorders are two main areas in which clinical studies are needed.

Original languageEnglish
Pages (from-to)563-573
Number of pages11
JournalExpert review of hematology
Volume12
Issue number7
DOIs
Publication statusPublished - Jul 2019

Keywords

  • Anemia, Sickle Cell/complications
  • Clinical Decision-Making
  • Disease Management
  • Disease Progression
  • Humans
  • Infections/etiology
  • Spleen/metabolism
  • Splenectomy
  • Splenomegaly/diagnosis

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