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Studies of Red‐Cell Membrane Function in Heterozygous β Thalassaemia and Other Hypochromic Anaemias
Author(s) -
KnoxMacaulay H. H. M.,
Weatherall D.J.
Publication year - 1974
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1974.tb00809.x
Subject(s) - red cell , endocrinology , medicine , cell , thalassemia , ouabain , chemistry , biochemistry , biology , sodium , organic chemistry
S ummary . Red‐cell membrane function in normal individuals has been compared with that of a group of patients with either heterozygous β thalassaemia, irondeficiency anaemia or sideroblastic anaemia. The metabolic abnormalities in β‐thalassaemic red cells were very similar to those observed in other forms of hypochromic anaemia. The hypochromic cells showed a marked increase in osmotic resistance after incubation for 24 hr which was corrected by buffered glucose but not by D‐xylose. Maximal inhibition of the Na + K‐ + membrane pumps by 10 ‐4 ouabain and 10 ‐3 M ethacrynic acid caused a significant increase in osmotic resistance of both normal and thalassaemic cells. The Na + and K + concentrations of ‘young’ and ‘old’ thalassaemic cell populations separated by centrifugation did not differ from normal. There was an increased rate of K + flux across the membrane of both thalassaemic and iron‐deficient red cells, with a net increase in the rate of K + loss from the cells. The rate of movement of Na + in and out of the cells was normal in all the hypochromic anaemias studied. The rates of glucose utilization and lactate production were increased in normal and thalassaemic ‘young’ cells but there was no increase in the ‘old’ populations of thalassaemic cells with increased K + fluxes. ATP levels were normal in all the abnormal cells studied. It is concluded that all the forms of hypochromic anaemia examined are characterized by increased K + fluxes and excessive loss of K + from the cells. The possible relationship between red‐cell haemoglobin content and membrane function is discussed.