title: Erythroblast- and erythrocyte-bound antibodies in alpha and beta thalassaemia syndromes.
abstract: Thirty-five Thai patients with various alpha-thalassaemia (alpha-thal 1/alpha-thal 2, alpha-thal 1/HbCS, HbCS/HbCS) and beta-thalassaemia (beta-thal/HbE, severe and mild form, HbE/HbE) syndromes were examined for the presence of immunoglobulins and C3d on o-tolidine positive erythroblasts in the bone marrow, and for the amounts of IgG of some specificities bound to circulating erythrocytes. In mild, but not in severe beta-thal/HbE and in alpha-thalassaemia, the percentages of Ig-positive erythroblasts were significantly higher than in controls and correlated well with the percentage of IgG-positive erythroblasts. By contrast, the percentages of IgM and C3d positive erythroblasts were low and similar in thalassaemic and control marrows. A substantial proportion of thalassaemic patients showed more erythrocyte-bound IgG than controls, but statistically significant elevations were seen only in severe beta-thal/HbE. Within a particular syndrome erythrocyte-bound IgG was more abundant in splenectomized than non-splenectomized subjects. It showed specificity for spectrin in some beta-thalassaemic patients and for band 3 protein in several individuals with alpha- or beta-thalassaemia. The results suggest that IgG antibodies play a role in the haemolysis of thalassaemia and that they are likely to be involved in the ineffective erythropoiesis in at least some of the syndromes studied.
authors.affiliation: Department of Haematology, St Mary's Hospital Medical School, Imperial College of Science, Technology and Medicine, London, U.K.
authors.forename: E
authors.initials: E
authors.lastname: Wiener
authors.forename: W
authors.initials: W
authors.lastname: Wanachiwanawin
authors.forename: K
authors.initials: K
authors.lastname: Kotipan
authors.forename: S
authors.initials: S
authors.lastname: Fucharoen
authors.forename: P
authors.initials: P
authors.lastname: Wasi
authors.forename: S N
authors.initials: SN
authors.lastname: Wickramasinghe
mesh_terms: D001457:Anion Exchange Protein 1, Erythrocyte / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology*; D015933:Complement C3d / Q000276:immunology; D004900:Erythroblasts / Q000166:cytology / Q000276:immunology*; D004912:Erythrocytes / Q000166:cytology / Q000276:immunology*; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology; D013049:Spectrin / Q000276:immunology; D013577:Syndrome; D017085:alpha-Thalassemia / Q000097:blood / Q000276:immunology*; D017086:beta-Thalassemia / Q000097:blood / Q000276:immunology*
chemical_list: D001457:Anion Exchange Protein 1, Erythrocyte; D001323:Autoantibodies; D007074:Immunoglobulin G; D013049:Spectrin; D015933:Complement C3d