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01-01-2004 | Original Article

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

Authors: D. M. Bogusławska, E. Heger, A. Chorzalska, M. Nierzwicka, J. Hołojda, A. Świderska, A. Straburzyńska, G. Paździor, M. Langner, A. F. Sikorski

Published in: Annals of Hematology | Issue 1/2004

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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Title: Hereditary spherocytosis: identification of several HS families with ankyrin and band 3 deficiency in a population of southwestern Poland
Authors: D. M. Bogusławska
E. Heger
A. Chorzalska
M. Nierzwicka
J. Hołojda
A. Świderska
A. Straburzyńska
G. Paździor
M. Langner
A. F. Sikorski
Publication date: 01-01-2004
Publisher: Springer-Verlag
Published in: Annals of Hematology / Issue 1/2004
Print ISSN: 0939-5555
Electronic ISSN: 1432-0584
DOI: https://doi.org/10.1007/s00277-003-0739-5

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