Hemolysis-associated pulmonary hypertension in thalassemia

Claudia R. Morris, Frans A. Kuypers, Gregory J. Kato, Lisa Lavrisha, Sandra Larkin, Titi Singer, Elliott P. Vichinsky

Research output: Contribution to journalArticlepeer-review

78 Scopus citations


Accumulating evidence supports the existence of a condition involving hemolysis-associated pulmonary hypertension (PUT). Hemolysis-induced release of cell-free hemoglobin and red blood cell arginase, resulting in impaired nitric oxide bioavailability, endothelial dysfunction, and PHT, has been reported in sickle cell disease. Since thalassemia is also a condition of chronic hemolysis, these patients are at risk. The data demonstrate that hemolysis-induced dysregulation of arginine metabolism and PHT also occurs in thalassemia. Erythrocyte release of arginase during hemolysis contributes to the development of PHT. Therapies that maximize arginine and nitric oxide bioavailability may benefit patients with thalassemia.

Original languageEnglish (US)
Pages (from-to)481-485
Number of pages5
JournalAnnals of the New York Academy of Sciences
StatePublished - 2005
Externally publishedYes


  • Hemolysis
  • Nitric oxide
  • Sickle cell disease
  • Thalassemia

ASJC Scopus subject areas

  • Neuroscience(all)
  • Biochemistry, Genetics and Molecular Biology(all)
  • History and Philosophy of Science


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