Abstract

IntroductionSickle cell anaemia (SCA) is an inherited, autosomal recessive condition that results from a mutation in the β-globin gene. Vascular occlusion is the underlying mechanism behind a myriad of complications encountered. This vascular occlusion is primarily caused by the increased tendency of red blood cells (RBC) to adhere to the vascular endothelium, and the activation of platelets and total leucocyte count (TLC), hence the need for a steady-state haematological profile in these patients.MethodThis was a cross-sectional study conducted over four months at a sickle cell clinic. Haemoglobin (Hb) concentration, haematocrit, platelet, TLC, mean corpuscular volume (MCV), mean corpuscular haemoglobin (MCH) and mean corpuscular haemoglobin concentration (MCHC) of the subjects were recorded and analysed.ResultsNinety-nine subjects aged 1-18 years were recruited for the study. There were 53 (53.5%) males. Leucocytosis was seen in 80 (80.8%), anaemia in 99 (100%), and thrombocytosis in 30 (30.3%) patients. The mean Hb, TLC and platelets were 7.9 ± 1.3g/dl, 14.3 ± 4.5 x 103/mm3 and 391.5 ± 182.6 x 103/mm3 respectively. Mean MCV, MCH and MCHC were 81.3 ± 7.1 fl, 28.6 ± 2.9 pg and 35.2 ± 1.7 g/dl respectively. Children aged one to four years had the highest TLC (p=0.002) but the lowest mean Hb and platelet (p=0.094 and 0.06) respectively. The mean MCV, MCH and MCHC were lowest in children aged one to four years (p=0.047, 0.001 and 0.001).ConclusionAnaemia, leucocytosis and thrombocytosis are characteristics features of children with SCA, especially in male and younger subjects. Although Iron markers are generally normal in children with SCA, those under the age of five years tend to have lower values.

Highlights

  • Sickle cell anaemia (SCA) is an inherited, autosomal recessive condition that results from a mutation in the β-globin gene

  • This vascular occlusion is primarily caused by the increased tendency of red blood cells (RBC) to adhere to the vascular endothelium, and the activation of platelets and total leucocyte count (TLC), the need for a steady-state haematological profile in these patients

  • The mean mean corpuscular volume (MCV), mean corpuscular haemoglobin (MCH) and mean corpuscular haemoglobin concentration (MCHC) were lowest in children aged one to four years (p=0.047, 0.001 and 0.001)

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Summary

Introduction

Sickle cell anaemia (SCA) is an inherited, autosomal recessive condition that results from a mutation in the β-globin gene. Vascular occlusion is the underlying mechanism behind a myriad of complications encountered. This vascular occlusion is primarily caused by the increased tendency of red blood cells (RBC) to adhere to the vascular endothelium, and the activation of platelets and total leucocyte count (TLC), the need for a steady-state haematological profile in these patients

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Disclosures
Powars DR
Iheanacho OE
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