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Comparisons of homozygous sickle cell disease in Northern Greece and Jamaica

Comparisons of homozygous sickle cell disease in Northern Greece and Jamaica
Comparisons of homozygous sickle cell disease in Northern Greece and Jamaica
The clinical and haematological features of homozygous sickle cell (SS) disease were compared in 30 Greek and 310 Jamaican patients. Deletional ?-thalassaemia, which modifies SS disease, is rare among Greek patients, so only Jamaican patients with four ?-globin genes were included in the control group. Greek patients had higher total haemoglobin concentration and red cell counts, and lower mean cell haemoglobin concentration (MCHC) and reticulocyte counts. They also had a more normal body build and more adults had persistent splenomegaly. Fewer had a history of leg ulceration or priapism but more reported acute chest syndrome. The comparatively mild disease in Greek patients is consistent with less haemolysis and sickling and therefore less bone marrow expansion. In the absence of amelioriating factors such as high HbF concentration or ?-thalassaemia, these findings may be explained by the low MCHC
0140-6736
637-640
Christakis, J.
3373319d-9e78-48ea-9c30-2e56170744a5
Vavatsi, N.
02e09047-a876-467e-bdfd-d755ca2f63e3
Hassapopoulou, H.
2159d7c9-fab7-4439-afa3-0492778ec06f
Papadopoulou, M.
ef30f6b5-b408-4656-8cff-a5c29c607bae
Mandreveli, K.
1e8eb5fd-bac6-464d-8a1a-f485384e20dc
Loukopoulos, D.
81759de6-773b-4429-bc22-3639fde8dd83
Morris, J.S.
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Serjeant, B.E.
d6cf580b-27e4-4943-9696-ddf4e51b9999
Serjeant, G.R.
0ee3a15b-b982-4b24-adbf-c1b0ebb65b71
Christakis, J.
3373319d-9e78-48ea-9c30-2e56170744a5
Vavatsi, N.
02e09047-a876-467e-bdfd-d755ca2f63e3
Hassapopoulou, H.
2159d7c9-fab7-4439-afa3-0492778ec06f
Papadopoulou, M.
ef30f6b5-b408-4656-8cff-a5c29c607bae
Mandreveli, K.
1e8eb5fd-bac6-464d-8a1a-f485384e20dc
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, N., Hassapopoulou, H., Papadopoulou, M., Mandreveli, K., Loukopoulos, D., Morris, J.S., Serjeant, B.E. and Serjeant, G.R. (1990) Comparisons of homozygous sickle cell disease in Northern Greece and Jamaica. The Lancet, 335 (8690), 637-640. (doi:10.1016/0140-6736(90)90419-6). (PMID:1690325)

Record type: Article

Abstract

The clinical and haematological features of homozygous sickle cell (SS) disease were compared in 30 Greek and 310 Jamaican patients. Deletional ?-thalassaemia, which modifies SS disease, is rare among Greek patients, so only Jamaican patients with four ?-globin genes were included in the control group. Greek patients had higher total haemoglobin concentration and red cell counts, and lower mean cell haemoglobin concentration (MCHC) and reticulocyte counts. They also had a more normal body build and more adults had persistent splenomegaly. Fewer had a history of leg ulceration or priapism but more reported acute chest syndrome. The comparatively mild disease in Greek patients is consistent with less haemolysis and sickling and therefore less bone marrow expansion. In the absence of amelioriating factors such as high HbF concentration or ?-thalassaemia, these findings may be explained by the low MCHC

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Published date: 1990
Organisations: Faculty of Health Sciences

Identifiers

Local EPrints ID: 365100
URI: https://eprints.soton.ac.uk/id/eprint/365100
ISSN: 0140-6736
PURE UUID: 4432450e-b8d4-4722-982e-c3a7c52586a6

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Date deposited: 29 May 2014 10:22
Last modified: 05 Oct 2018 12:09

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Contributors

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

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