Association of soluble fms-like tyrosine kinase-1 with pulmonary hypertension and haemolysis in sickle cell disease

Kenneth I. Ataga, Julia Elizabeth Brittain, Susan K. Jones, Ryan May, John Delaney, Dell Strayhorn, Payal Desai, Rupa Redding-Lallinger, Nigel S. Key, Eugene P. Orringer

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17 Scopus citations

Abstract

The pathophysiology of pulmonary hypertension (PHT) in sickle cell disease (SCD) is probably multifactorial. Soluble fms-like tyrosine kinase-1 (sFLT-1) is a member of the vascular endothelial growth factor receptor (VEGFR) family. By adhering to and inhibiting VEGF and placenta growth factor, it induces endothelial dysfunction. We sought to evaluate the association of sFLT-1 with clinical complications of SCD. We confirmed that sFLT-1 was significantly elevated in SCD patients compared to healthy, race-matched control subjects. The level of sFLT-1 was significantly higher in patients with PHT, but no association was observed between sFLT-1 and the frequency of acute pain episodes or history of acute chest syndrome. sFLT-1 was correlated with various measures of haemolysis, erythropoietin and soluble vascular cell adhesion molecule-1. By inducing endothelial dysfunction, sFLT-1 may contribute to the pathogenesis of SCD-associated PHT, although this effect does not appear to be independent of haemolysis.

Original languageEnglish (US)
Pages (from-to)485-491
Number of pages7
JournalBritish Journal of Haematology
Volume152
Issue number4
DOIs
StatePublished - Feb 1 2011

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Keywords

  • Endothelial activation
  • Haemolysis
  • Pulmonary hypertension
  • Sickle cell disease
  • Soluble fms-like tyrosine kinase-1 (sFLT-1)

ASJC Scopus subject areas

  • Hematology

Cite this

Ataga, K. I., Brittain, J. E., Jones, S. K., May, R., Delaney, J., Strayhorn, D., Desai, P., Redding-Lallinger, R., Key, N. S., & Orringer, E. P. (2011). Association of soluble fms-like tyrosine kinase-1 with pulmonary hypertension and haemolysis in sickle cell disease. British Journal of Haematology, 152(4), 485-491. https://doi.org/10.1111/j.1365-2141.2010.08410.x