Haematological change in sickle cell–haemoglobin C disease and in sickle cell-beta thalassaemia: a cohort study from birth

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Abstract
The hematological changes in early years following neonatal diagnosis were observed in representative groups of children with sickle cell-hemoglobin C (SC) disease, sickle cell-.beta.+ thalassemia, and in sickle cell-.beta.0 thalassemia. Most hematological 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 Hb concentration from age 2 mo. to 4 yr. The combination of a raised MCHC [mean corpuscular hemaglobin concentration] and a lowered MCV [mean corpuscular volume] is unusual and may be characteristic of SC disease. Features in sickle cell-.beta. thalassemia generally differed according to the type of .beta. thalassemia gene. Sickle cell-.beta.0 thalassemia had lower levels of Hb, MCHC, red cell count, MCV and higher reticulocytes, most differences being significant before 1 yr. No differences between S.beta.0 thalassemia and S.beta.+ thalassemia were apparent in HbF levels (which resembled those in SS disease) or in HbA2 levels (which exceeded those in SS disease by 1 yr of age).