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Hereditary spherocytosis: identification of several HS families with ankyrin and band 3 deficiency in a population of southwestern Poland

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Abstract

Red blood cells of 17 patients out of seven families diagnosed with HS from the southwest of Poland were studied. In six families a deficiency of ankyrin was detected, and in one family a band 3 (anion-exchanger protein) deficiency was detected. Patients from six families with the ankyrin deficiency had a 19–51% decrease in ankyrin 2.1, while the family with the band 3 deficiency showed a 33% decrease in this protein content. All changes were statistically significant, as analysed by the Student t test (P<0.05). Analysis of haemolysis kinetics gives a reliable indication of altered osmotic properties of the spherocytic cells.

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Authors and Affiliations

  1. Institute of Biochemistry and Molecular Biology, University of Wrocław, ul. Przybyszewskiego 63/77, 51-148, Wrocław, Poland

    A. Chorzalska & A. F. Sikorski

  2. Institute of Biotechnology and Environmental Sciences, University of Zielona Góra, ul. Monte Cassino 21B, 65-561, Zielona Góra, Poland

    D. M. Bogusławska, E. Heger, M. Nierzwicka & A. F. Sikorski

  3. Division of Haematology and Blood Oncology, Regional Hospital, ul. Iwaszkiewicza 5, 59-220, Legnica, Poland

    J. Hołojda

  4. MSWiA Infirmary, ul. Wazów 42, 65-001, Zielona Góra, Poland

    A. Świderska

  5. Regional Hospital, ul. Zyty 26, 65-001, Zielona Góra, Poland

    A. Straburzyńska

  6. Institute of Physics, Wrocław University of Technology, Wybrzeże Wyspiańskiego 27, 50-370, Wrocław, Poland

    G. Paździor & M. Langner

Authors
  1. D. M. Bogusławska
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  2. E. Heger
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  3. A. Chorzalska
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  4. M. Nierzwicka
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  5. J. Hołojda
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  6. A. Świderska
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  7. A. Straburzyńska
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  8. G. Paździor
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  9. M. Langner
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  10. A. F. Sikorski
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Corresponding author

Correspondence to A. F. Sikorski.

Additional information

Portions of these results have been previously presented in an abstract form at the VIII Polish Conference on Cell Biology, Wrocław, 2002 [1]

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Bogusławska, D.M., Heger, E., Chorzalska, A. et al. Hereditary spherocytosis: identification of several HS families with ankyrin and band 3 deficiency in a population of southwestern Poland. Ann Hematol 83, 28–33 (2004). https://doi.org/10.1007/s00277-003-0739-5

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  • DOI: https://doi.org/10.1007/s00277-003-0739-5

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