Pleiotropic effects of intravascular haemolysis on vascular homeostasis

Gregory J. Kato, James G. Taylor VI

Research output: Contribution to journalReview articlepeer-review

55 Scopus citations


The breakdown of senescent or defective red blood cells releases red cell contents, especially haemoglobin, which scavenges nitric oxide (NO) and decomposes to haem and free iron. These are potent oxidants, all of which have promoted the evolution of inducible and vasculoprotective compensatory pathways to rapidly clear and detoxify haemoglobin, haem and iron. Chronic haemolytic red cell disorders as diverse as sickle cell disease, thalassaemia, unstable haemoglobinopathy, cytoskeletal defects and enzymopathies have been linked to a clinical constellation of pulmonary hypertension, priapism, leg ulceration and possibly cerebrovascular disease and thrombosis. Besides free haemoglobin, haemolysis has been associated with extracellular arginase that limits substrate availability to NO synthase, endogenous inhibitors of NO synthase activity, and inappropriate activation of haemostatic pathways. This article reviews the haemolytic disorders that have been reported to manifest vascular complications, and explores the speculative possibility that haemolysis mediates some of the vascular complications of inflammation and diabetes.

Original languageEnglish (US)
Pages (from-to)690-701
Number of pages12
JournalBritish journal of haematology
Issue number5
StatePublished - Mar 2010
Externally publishedYes


  • Haemolysis
  • Nitric oxide
  • Sickle cell disease
  • Thalassaemia
  • Vasculopathy

ASJC Scopus subject areas

  • Hematology


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