Improvement in hemolysis and pulmonary arterial systolic pressure in adult patients with sickle cell disease during treatment with hydroxyurea

Matthew Olnes, Amy Chi, Carissa Haney, Rose May, Caterina Minniti, Taylor James VI, Gregory J. Kato

Research output: Contribution to journalArticle

Abstract

Elevated pulmonary arterial systolic pressure is strongly associated with mortality in patients with sickle cell disease (SCD). A tricuspid regurgitant velocity (TRV) of 2.5 m/s or greater by trans-thoracic echocardiogram is a key marker of risk [1-3]. The pathophysiologic mechanism involves release from the red cell during intravascular hemolysis of cell-free plasma hemoglobin and arginase [4]. Hydroxyurea is the only drug approved by the Food and Drug Administrations specifically for SCD. It acts by increasing levels of fetal hemoglobin, which inhibits sickling, and has been shown to reduce the incidence of vaso-occlusive crisis (VOC), and prolong survival in patients with sickle cell disease [5,6]. Because fetal hemoglobin also reduces the rate of hemolysis in SCD, hypothetically, hydroxyurea might also reduce the severity of hemolysis-linked vascular dysfunction and pulmonary hypertension. Herein, we describe five patients with sickle cell disease having elevated pulmonary arterial systolic pressure who exhibited improvement in their baseline laboratory parameters of hemolysis, accompanied by reduced TRV, during treatment with hydroxyurea. Hydroxyurea may have a role in the management of selected patients with elevated TRV.

Original languageEnglish (US)
Pages (from-to)530-532
Number of pages3
JournalAmerican Journal of Hematology
Volume84
Issue number8
DOIs
StatePublished - Aug 2009
Externally publishedYes

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Hydroxyurea
Sickle Cell Anemia
Hemolysis
Arterial Pressure
Blood Pressure
Lung
Fetal Hemoglobin
Therapeutics
Arginase
United States Food and Drug Administration
Plasma Cells
Pulmonary Hypertension
Blood Vessels
Hemoglobins
Thorax
Survival
Mortality
Incidence
Pharmaceutical Preparations

ASJC Scopus subject areas

  • Hematology

Cite this

Improvement in hemolysis and pulmonary arterial systolic pressure in adult patients with sickle cell disease during treatment with hydroxyurea. / Olnes, Matthew; Chi, Amy; Haney, Carissa; May, Rose; Minniti, Caterina; James VI, Taylor; Kato, Gregory J.

In: American Journal of Hematology, Vol. 84, No. 8, 08.2009, p. 530-532.

Research output: Contribution to journalArticle

Olnes, Matthew ; Chi, Amy ; Haney, Carissa ; May, Rose ; Minniti, Caterina ; James VI, Taylor ; Kato, Gregory J. / Improvement in hemolysis and pulmonary arterial systolic pressure in adult patients with sickle cell disease during treatment with hydroxyurea. In: American Journal of Hematology. 2009 ; Vol. 84, No. 8. pp. 530-532.
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