Vascular complications of sickle cell disease

Ashar Usmani, Roberto F. Machado

Research output: Contribution to journalArticle

2 Scopus citations

Abstract

Sickle cell disease (SCD) is a monogenetic disorder caused by a mutation in the β-globin gene HBB leading to polymerization of red blood cells causing damage to cell membranes, increasing its rigidity and intravascular hemolysis. Multiple lines of evidence suggest that SCD can be viewed as pan-vasculopathy associated with multiple mechanisms but driven by hemoglobin S polymerization. Here we review the pathophysiology, clinical manifestations and management strategies for cerebrovascular disease, pulmonary hypertension and renal disease associated with SCD. These "vascular phenotypes" reflect the systemic nature of the complications of SCD and are a major threat to the well-being of patients with the disorder.

Original languageEnglish (US)
Pages (from-to)205-221
Number of pages17
JournalClinical hemorheology and microcirculation
Volume68
Issue number2-3
DOIs
StatePublished - Jan 1 2018

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Keywords

  • Sickle cell disease
  • cerebral vasculopathy
  • nitric oxide
  • pulmonary hypertension
  • renal disease

ASJC Scopus subject areas

  • Physiology
  • Hematology
  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

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