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Hemolysis‐Associated Pulmonary Hypertension in Thalassemia
Author(s) -
MORRIS CLAUDIA R.,
KUYPERS FRANS A.,
KATO GREGORY J.,
LAVRISHA LISA,
LARKIN SANDRA,
SINGER TITI,
VICHINSKY ELLIOTT P.
Publication year - 2005
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1196/annals.1345.058
Subject(s) - hemolysis , thalassemia , pulmonary hypertension , medicine , beta thalassemia , cardiology
A bstract : Accumulating evidence supports the existence of a condition involving hemolysis‐associated pulmonary hypertension (PHT). 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.