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A comparison of sickle cell syndromes in Northern Greece

A comparison of sickle cell syndromes in Northern Greece
A comparison of sickle cell syndromes in Northern Greece
Haematological and clinical characteristics have been examined in 30 patients with homozygous sickle cell (SS) disease, 28 with sickle cell-beta° thalassaemia, and 21 with sickle cell-beta+ thalassaemia. The latter could be divided into three groups on their molecular basis and HbA levels, four subjects with an IVS-2 nt 745 mutation having 34% HbA (designated Sbeta+ thalassaemia type I), 14 subjects with an IVS-1 nt 110 mutation having 8–15% HbA (designated Sbeta+ thalassaemia type 11). and three subjects with an IVS–1 nt 6 mutation having 20–25% HbA (designated Sbeta+ thalassaemia type III). Comparisons were conducted between SS disease, Sbeta° thalassaemia, and Sbeta+ thalassaemia type II. Compared to SS disease, both thalassaemia syndromes had higher HbAr levels and red cell counts and lower mean cell haemoglobin content (MCHC), mean cell volume (MCV) and MCH, and Sbeta° thalassaemia had higher HbF and reticulocyte counts. Compared to Sbeta° thalassaemia. Sbeta+ thalassaemia had a higher haemoglobin and MCHC. Clinically, persistence of splenomegaly was more common in Sbeta° and Sbeta+ thalassaemia type II compared to SS disease. Few significant differences occurred between SS disease, Sbeta° and Sbeta+ thalassaemia type II in Northern Greece suggesting that the 8–15% HbA in the latter condition was insufficient to modify the clinical course
0007-1048
386-391
Christakis, J.
3373319d-9e78-48ea-9c30-2e56170744a5
Vavatsi, M.
ab496c09-94fd-4e0c-84e0-920b5d353a9e
Hassapopoulou, H.
2159d7c9-fab7-4439-afa3-0492778ec06f
Papadopoulou, M.
ef30f6b5-b408-4656-8cff-a5c29c607bae
Loukopoulos, D.
81759de6-773b-4429-bc22-3639fde8dd83
Morris, J.S.
569aa43b-15bd-4e9d-b4a5-e68a84334cfe
Serjeant, B.E.
d6cf580b-27e4-4943-9696-ddf4e51b9999
Serjeant, G.R.
0ee3a15b-b982-4b24-adbf-c1b0ebb65b71
Christakis, J.
3373319d-9e78-48ea-9c30-2e56170744a5
Vavatsi, M.
ab496c09-94fd-4e0c-84e0-920b5d353a9e
Hassapopoulou, H.
2159d7c9-fab7-4439-afa3-0492778ec06f
Papadopoulou, M.
ef30f6b5-b408-4656-8cff-a5c29c607bae
Loukopoulos, D.
81759de6-773b-4429-bc22-3639fde8dd83
Morris, J.S.
569aa43b-15bd-4e9d-b4a5-e68a84334cfe
Serjeant, B.E.
d6cf580b-27e4-4943-9696-ddf4e51b9999
Serjeant, G.R.
0ee3a15b-b982-4b24-adbf-c1b0ebb65b71

Christakis, J., Vavatsi, M., Hassapopoulou, H., Papadopoulou, M., Loukopoulos, D., Morris, J.S., Serjeant, B.E. and Serjeant, G.R. (1991) A comparison of sickle cell syndromes in Northern Greece. British Journal of Haematology, 77 (3), 386-391. (doi:10.1111/j.1365-2141.1991.tb08589.x). (PMID:2012764)

Record type: Article

Abstract

Haematological and clinical characteristics have been examined in 30 patients with homozygous sickle cell (SS) disease, 28 with sickle cell-beta° thalassaemia, and 21 with sickle cell-beta+ thalassaemia. The latter could be divided into three groups on their molecular basis and HbA levels, four subjects with an IVS-2 nt 745 mutation having 34% HbA (designated Sbeta+ thalassaemia type I), 14 subjects with an IVS-1 nt 110 mutation having 8–15% HbA (designated Sbeta+ thalassaemia type 11). and three subjects with an IVS–1 nt 6 mutation having 20–25% HbA (designated Sbeta+ thalassaemia type III). Comparisons were conducted between SS disease, Sbeta° thalassaemia, and Sbeta+ thalassaemia type II. Compared to SS disease, both thalassaemia syndromes had higher HbAr levels and red cell counts and lower mean cell haemoglobin content (MCHC), mean cell volume (MCV) and MCH, and Sbeta° thalassaemia had higher HbF and reticulocyte counts. Compared to Sbeta° thalassaemia. Sbeta+ thalassaemia had a higher haemoglobin and MCHC. Clinically, persistence of splenomegaly was more common in Sbeta° and Sbeta+ thalassaemia type II compared to SS disease. Few significant differences occurred between SS disease, Sbeta° and Sbeta+ thalassaemia type II in Northern Greece suggesting that the 8–15% HbA in the latter condition was insufficient to modify the clinical course

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Published date: 1991
Organisations: Faculty of Health Sciences

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Local EPrints ID: 365113
URI: http://eprints.soton.ac.uk/id/eprint/365113
ISSN: 0007-1048
PURE UUID: 9fe3510e-f677-4a6f-895d-7e560f11eb84

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Date deposited: 22 May 2014 11:22
Last modified: 14 Mar 2024 16:46

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Contributors

Author: J. Christakis
Author: M. Vavatsi
Author: H. Hassapopoulou
Author: M. Papadopoulou
Author: D. Loukopoulos
Author: J.S. Morris
Author: B.E. Serjeant
Author: G.R. Serjeant

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