Chronic sickle cell lung disease: New insights into the diagnosis, pathogenesis and treatment of pulmonary hypertension

Roberto F. Machado, Mark T. Gladwin

Research output: Contribution to journalReview articlepeer-review

92 Scopus citations


Pulmonary hypertension is a common complication of sickle cell disease (SCD). In spite of the mild elevations in pulmonary artery pressures in these patients, the associated morbidity and mortality is high. In fact, in adult patients with SCD, pulmonary hypertension is emerging as the major independent risk factor for death. The aetiology of pulmonary hypertension is probably multifactorial, including haemolysis, impaired nitric oxide bioavailability, chronic hypoxaemia, thromboembolism, parenchymal and vascular injury because of sequestration of sickle erythrocytes, chronic liver disease and asplenia. Interestingly, pulmonary hypertension is emerging as a common, and probably, invariant sequella of lifelong haemolytic anaemia in other hereditary and acquired haemolytic diseases, such as thalassaemia, stomatocytosis and spherocytosis. There are currently limited specific data on the effects of any treatment modality for pulmonary hypertension in patients with SCD. It is likely that maximization of SCD therapy, in all patients, and treatment with selective pulmonary vasodilators and antiproliferative agents, in patients with severe disease, would be beneficial. A large trial evaluating the effects of therapy for pulmonary hypertension in the SCD population is clearly indicated.

Original languageEnglish (US)
Pages (from-to)449-464
Number of pages16
JournalBritish journal of haematology
Issue number4
StatePublished - May 2005


  • Haemolysis
  • Nitric oxide
  • Pulmonary hypertension
  • Sickle cell disease

ASJC Scopus subject areas

  • Hematology

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