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Ann Hematol ; 83(1): 28-33, 2004 Jan.
Article in English | MEDLINE | ID: mdl-14517693

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.


Subject(s)
Anion Exchange Protein 1, Erythrocyte/deficiency , Ankyrins/blood , Spherocytosis, Hereditary/blood , Adolescent , Adult , Ankyrins/deficiency , Blood Protein Electrophoresis , Child , Erythrocyte Membrane/metabolism , Erythrocytes/chemistry , Family , Hemolysis , Humans , Middle Aged , Poland
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