Haematological Profile of Children With Sickle Cell Anaemia in Steady State

Abstract

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), hence the need for a steady-state haematological profile in these patients. Method This 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. Results Ninety-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 10 3 /mm 3 and 391.5 ± 182.6 x 10 3 /mm 3 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). Conclusion Anaemia, 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.

Hematological Characteristics of a Sample of Iraqi Patients with Sickle Cell Anemia and Their Correlation with Age and Gender

“…In addition, age of patients did not affect hemoglobin levels significantly. These findings are inconsistent with the observation of previous authors [14] who described low hemoglobin levels in young children and in male patients. Low hemoglobin levels in young children have been also reported by Iheanacho et al [18] and low hemoglobin levels in males have been reported by Abubakar et al [19].…”

“…In the current study, the mean WBC count of patients was within normal and most patients had normal WBC counts; however, leukopenia was more frequently seen in females than in males and leukocytosis was reported in 2 cases only. Actually, these findings disagree with the findings of previous authors [14,18,19] who found significant leukocytosis in patients with sickle cell anemia. It has been suggested that steady state of inflammation in patients with sickle cell anemia could explain the rise in WBC count associated with cytokine production [24].…”

“…These indices reflect normal iron status, which is the result of increased red cell turn-over and blood transfusion in patients with sickle cell disease. Moreover, the low MCV and MCH in males in our study is similar to the observation of some previous authors [14].…”

“…Actually, the low hemoglobin levels of nearly all patients enrolled in this study are in line with the observation of previous studies [14 -17]. The reasons for low hemoglobin in patients with sickle cell disease are attributed to nutritional insufficiency, low erythropoietin response and the most important "reduced life span of RBC" [14][15][16][17].…”

hematological parameters levels study in sickle cell anemia patients in Al-Diwaniyah and Al-Najaf governorates

“…Many studies documented that increased in WBC sickle cell anemia patients such as Harp et al (2021), Ahmed et al,(2017) and Akinbami et al (2012), and this is fully agrees with the results of the current study.The present results indicated a significant increase in PLT in sickle cell anemia patients compared with control group. That's agree with the results of the study ofAliu et al,(2020) andOlaniyi et al,(2014) who reported One-third of those with SCA had thrombocytosis, or an increased platelet count. Thrombocytosis in SCA has a number of different causes, including anemia-induced increased erythropoietin secretion, which has homology with thrombopoietin and leads to thrombopoiesis, as well as functional and/or structural asplenia, which is a feature of SCA.On the other hand, the results of the current study agree with the results of the study of Aliyu et al (2008), Antwi-Boasiako et al (2018) and Akwiwu et al (2020) who reported the higher platelet involvement was caused by more frequent crises and more years of life with sickle cell anemia.…”