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F1000 Med Rep. 2010 Feb 11;2. pii: 10. doi: 10.3410/M2-10.

Pulmonary hypertension in hemolytic anemias.

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  • 1Department of Hematology/Oncology, Children's Hospital and Research Center Oakland 747 52nd Street, Oakland, CA 94609 USA.

Abstract

Pulmonary hypertension (PH) has been reported with nearly all forms of the inherited as well as the acquired hemolytic anemias. Recent research investigating the pathophysiology of PH in sickle cell disease and thalassemia has helped elucidate the central role of hemolysis-mediated endothelial dysfunction in the development of PH in these populations. Although the most appropriate treatment of PH in patients with hemolytic anemia is not clearly defined, the associated significant increased risk of death underscores the need for randomized clinical trials in this area.

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