Red cell deformability, splenic function and anaemia in thalassaemia

Authors

  • A. M. Dondorp,

    1. Department of Internal Medicine and Division of Infectious Diseases, Tropical Medicine and AIDS, Academic Medical Centre, Amsterdam, The Netherlands,
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  • K. T. Chotivanich,

    1. Faculty of Tropical Medicine, Mahidol University, Bangkok, Thailand,
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  • S. Fucharoen,

    1. Thalassaemia Research Centre, Institute of Science and Technology for Research and Development, Division of Haematology, Department of Medicine, Mahidol University, Bangkok, Thailand,
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  • K. Silamut,

    1. Faculty of Tropical Medicine, Mahidol University, Bangkok, Thailand,
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  • J. Vreeken,

    1. Department of Internal Medicine and Division of Infectious Diseases, Tropical Medicine and AIDS, Academic Medical Centre, Amsterdam, The Netherlands,
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  • P. A. Kager,

    1. Department of Internal Medicine and Division of Infectious Diseases, Tropical Medicine and AIDS, Academic Medical Centre, Amsterdam, The Netherlands,
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  • N. J. White

    1. Faculty of Tropical Medicine, Mahidol University, Bangkok, Thailand,
    2. Centre for Tropical Medicine, Nuffield Department of Tropical Medicine, John Radcliffe Hospital, Oxford, U.K.
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Dr A. M. Dondorp Department of Internal Medicine and Division of Infectious Diseases, Tropical Medicine and AIDS, Academic Medical Centre, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.

Abstract

Red cell deformability (RCD) was measured in 38 patients with α-thalassaemia and 48 patients with β-thalassaemia, of whom 13 had undergone splenectomy. All splenectomized patients, but none of those with intact spleens, had very rigid erythrocytes with an elongation index <0.45 at a high shear stress of 30 Pa suggesting a splenic recognition threshold for removal of rigid red cells. At this shear stress RCD correlated strongly with the degree of anaemia in both the splenectomized (r = 0.81, P < 0.001) and non-splenectomized β-thalassaemic patients (all patients r = 0.81, P < 0.001; homozygous β-thalassaemic patients r = 0.51, P = 0.01). These data suggest that reduced RCD is a major determinant of anaemia in thalassaemia.

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