Abstract:
:The haematological changes in early years following neonatal diagnosis have been observed in representative groups of children with sickle cell-haemoglobin C (SC) disease, sickle cell-beta(+) thalassaemia, and in sickle cell-beta(0) thalassaemia. Most haematological indices in SC disease were intermediate between previously published values in SS disease and in AA controls, generally being closer to values in normal children. Exceptions were microcytosis which may be genetically determined and a striking elevation of mean cell haemoglobin concentration from age 2 months to 4 years. The combination of a raised MCHC and a lowered MCV is unusual and may be characteristic of SC disease. Features in sickle cell-beta thalassaemia generally differed according to the type of beta thalassaemia gene. Sickle cell-beta(0) thalassaemia had lower levels of haemoglobin, MCHC, red cell count, MCV, and higher reticulocytes, most differences being significant before 1 year. No differences between S beta(0) thalassaemia and S beta(+) thalassaemia were apparent in HbF levels (which resembled those in SS disease) or in HbA2 levels (which exceeded those in SS disease by 1 year of age).
journal_name
Br J Haematoljournal_title
British journal of haematologyauthors
Stevens MC,Maude GH,Beckford M,Grandison Y,Mason K,Serjeant BE,Taylor B,Topley JM,Serjeant GRdoi
10.1111/j.1365-2141.1985.tb07414.xsubject
Has Abstractpub_date
1985-06-01 00:00:00pages
279-92issue
2eissn
0007-1048issn
1365-2141journal_volume
60pub_type
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