Skeletal and myocardial microvascular blood flow in hydroxycarbamide-treated patients with sickle cell disease

Vandana Sachdev, Stanislav Sidenko, Melinda D. Wu, Caterina P. Minniti, Hwaida Hannoush, Cynthia L. Brenneman, Myron A. Waclawiw, Andrew E. Arai, Alan N. Schechter, Gregory J. Kato, Jonathan Lindner

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    9 Scopus citations


    In sickle cell disease (SCD), abnormal microvascular function combined with chronic anaemia predisposes patients to perfusion-demand mismatch. We hypothesized that skeletal muscle and myocardial perfusion, normalized to the degree of anaemia, is reduced at basal-state compared to controls, and that this defect is ameliorated by hydroxycarbamide (HC; also termed hydroxyurea) therapy. Twenty-one SCD patients, of whom 15 were treated with HC, and 27 controls underwent contrast-enhanced ultrasound (CEU) perfusion imaging of the forearm as well as the myocardium. HC treatment was associated with lower white cell and reticulocyte counts, and higher fetal haemoglobin and total haemoglobin levels. When corrected for the degree of anaemia in SCD patients, skeletal flow in HC-treated patients was significantly higher than in untreated SCD patients (217·7 ± 125·4 vs. 85·9 ± 40·2, P = 0·018). Similarly, when normalized for both anaemia and increased myocardial work, resting myocardial perfusion was also significantly higher in HC-treated patients compared with untreated SCD patients (0·53 ± 0·47 vs. 0·13 ± 0·07, P = 0·028). Haemoglobin F (HbF) levels correlated with skeletal muscle microvascular flow (r = 0·55, P = 0·01). In conclusion, patients with SCD not on HC therapy have resting flow deficits in both skeletal muscle and myocardial flow. HC therapy normalizes flow and there is a direct correlation with HbF levels. Clinical trial registration Identifier: NCT01602809;

    Original languageEnglish (US)
    JournalBritish Journal of Haematology
    StateAccepted/In press - 2017



    • Contrast ultrasound
    • Hydroxycarbamide
    • Microvasculature
    • Perfusion imaging
    • Sickle cell disease

    ASJC Scopus subject areas

    • Hematology

    Cite this

    Sachdev, V., Sidenko, S., Wu, M. D., Minniti, C. P., Hannoush, H., Brenneman, C. L., Waclawiw, M. A., Arai, A. E., Schechter, A. N., Kato, G. J., & Lindner, J. (Accepted/In press). Skeletal and myocardial microvascular blood flow in hydroxycarbamide-treated patients with sickle cell disease. British Journal of Haematology.