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

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).

First recorded case of haemoglobin SC in sudan

“…They had high WBC and RDW and low Hb, PCV and RBC count. This is the same as reported for HbSC by Stevens and his colleagues [22]. In contrast, Hb, PCV and RBC levels were low in their mother, but her RDW and WBC were normal ( Table 1).…”

Effect of Age on Blood Rheology in Sickle Cell Anaemia and Sickle Cell Haemoglobin C Disease: A Cross-Sectional Study

“…The evolution of several haematological indices, as well as foetal haemoglobin (HbF) level (a strong modulator of clinical severity in SS), have been well described in SCD populations from birth to adulthood and during ageing [ 13 , 14 , 15 ]. It has previously been shown that blood rheology may be influenced by age in the general population with blood viscosity and RBC aggregation increasing, and RBC deformability decreasing, with age [ 16 ].…”