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Erythrocyte membranes from a patient with congenital dyserythropoietic anaemia type I (CDA‐I) show identical, although less pronounced, glycoconjugate abnormalities to those from patients with CDA‐II (HEMPAS)
Author(s) -
Zdebska Ewa,
Woźniewicz Bohdan,
AdamowiczSalach Anna,
Kościelak Jerzy
Publication year - 2000
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 - 10.1046/j.1365-2141.2000.02288.x
Subject(s) - glycoconjugate , ineffective erythropoiesis , erythropoiesis , erythrocyte membrane , medicine , band 3 , biology , pathology , immunology , anemia , biochemistry , membrane
Congenital dyserythropoietic anaemias (CDAs) are rare hereditary disorders characterized by ineffective erythropoiesis and multinuclearity of erythroblasts. Three main types of the disease have been described. Glycoconjugate abnormalities in erythrocyte membrane glycoconjugates, consisting of hypoglycosylation of band 3 and accumulation of certain glycosphingolipids including lactotriaosylceramide, neolactotriaosylceramide and polyglycosylceramides, have been described only in patients with CDA type II (CDA‐II). We report on identical, although less pronounced, abnormalities in erythrocyte glycoconjugates from a patient with CDA‐I. A low degree of hypoglycosylation of band 3 in our patient with CDA‐I suggests that hypoglycosylation is not a cause, but, most probably, a consequence of dyserythropoiesis.