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Haematological change in sickle cell–haemoglobin C disease and in sickle cell‐beta thalassaemia: a cohort study from birth
Author(s) -
Stevens M. C. G.,
Maude G. H.,
Beckford M.,
Grandison Y.,
Mason K.,
Serjeant B. E.,
Taylor B.,
Topley J. M.,
Serjeant G. R.
Publication year - 1985
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 - 10.1111/j.1365-2141.1985.tb07414.x
Subject(s) - microcytosis , fetal hemoglobin , hemoglobinopathy , medicine , sickle cell anemia , beta thalassaemia , disease , acute chest syndrome , hemoglobin a2 , beta thalassemia , cohort , hemoglobin c , cell , gastroenterology , pediatrics , hemoglobin , anemia , thalassemia , biology , genetics , pregnancy , iron deficiency , fetus
S ummary 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‐β + thalassaemia, and in sickle cell‐β° 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‐β thalassaemia generally differed according to the type ofβ thalassaemia gene. Sickle cell‐β° thalassaemia had lower levels of haemoglobin, MCHC, red cell count, MCV, and higher reticulocytes, most differences being significant before 1 year. No differences between Sβ° thalassaemia and Sβ + thalassaemia were apparent in HbF levels (which resembled those in SS disease) or in HbA 2 levels (which exceeded those in SS disease by 1 year of age).