Pulmonary hypertension and nitric oxide depletion in sickle cell disease.

Published

Journal Article (Review)

During the past decade a large body of experimental and clinical studies has focused on the hypothesis that nitric oxide (NO) depletion by plasma hemoglobin in the microcirculation plays a central role in the pathogenesis of many manifestations of sickle cell disease (SCD), particularly pulmonary hypertension. We have carefully examined those studies and believe that the conclusions drawn from them are not adequately supported by the data. We agree that NO depletion may well play a role in the pathophysiology of other hemolytic states such as paroxysmal nocturnal hemoglobinuria, in which plasma hemoglobin concentrations are often at least an order of magnitude greater than in SCD. Accordingly, we conclude that clinical trials in SCD designed to increase the bioavailability of NO or association studies in which SCD clinical manifestations are related to plasma hemoglobin via its surrogates should be viewed with caution.

Full Text

Duke Authors

Cited Authors

  • Bunn, HF; Nathan, DG; Dover, GJ; Hebbel, RP; Platt, OS; Rosse, WF; Ware, RE

Published Date

  • August 2010

Published In

Volume / Issue

  • 116 / 5

Start / End Page

  • 687 - 692

PubMed ID

  • 20395414

Pubmed Central ID

  • 20395414

Electronic International Standard Serial Number (EISSN)

  • 1528-0020

International Standard Serial Number (ISSN)

  • 0006-4971

Digital Object Identifier (DOI)

  • 10.1182/blood-2010-02-268193

Language

  • eng