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Clinical and haematological diversity of sickle cell disease in Saudi children
1Department of Medical Biochemistry, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Insights
Sickle cell disease (SCD) shows varied severity in Saudi Arabia. Children in the south-western province experience more severe SCD with distinct clinical and hematological differences compared to those in the eastern province.
Area of Science:
- Hematology
- Genetics
- Pediatrics
Background:
- Sickle cell disease (SCD) presents a wide range of clinical severity.
- Understanding regional variations in SCD is crucial for effective management.
Purpose of the Study:
- To investigate the clinical and hematological diversity of SCD in different regions of Saudi Arabia.
- To compare SCD severity and manifestations between children from the Eastern Province and the South-Western Province (SWP).
Main Methods:
- Comparative analysis of hematological parameters and clinical data.
- Inclusion of 41 children with SCD from the Eastern Province and 51 from the SWP.
- Calculation of Severity Index (SI) and assessment of clinical manifestations.
Main Results:
- Patients from SWP exhibited a higher mean SI (9.5) compared to the Eastern Province (4.5).
- SWP patients showed higher incidence of hand/foot syndrome, vaso-occlusive crisis, and increased need for transfusions and hospitalization.
- Lower red blood cell count, total hemoglobin, and packed cell volume were observed in SWP patients; Hemoglobin A2 levels were higher.
Conclusions:
- Sickle cell disease in the Saudi population is clinically and hematologically heterogeneous.
- Regional differences significantly impact SCD presentation and severity.
- Further research into genetic and environmental factors influencing SCD heterogeneity is warranted.
Abstract:
Sickle cell disease (SCD) exhibits itself in a broad spectrum of clinical behaviour ranging from a mild disease to an incapacitating condition. In this study, we have attempted to investigate the clinical diversity of SCD in different regions of Saudi Arabia. The results of haematological parameters and clinical manifestations in 41 children with SCD from the eastern province where the disease is mild, were compared with results obtained in 51 children from the south-western province (SWP), where the disease has been shown to be more severe. The severity index (SI) of patients from the eastern province ranged from 2 to 11 with a mean of 4.5 and in patients from the south-western province, the SI ranged from 2 to 18 with a mean of 9.5. In addition, the occurrence of hand and foot syndrome, vaso-occlusive crisis, and increased frequency of requirement of blood transfusion and hospitalization differentiated the clinical presentation of SCD in the patients from south-western province from those in the eastern province. Red blood cell level, total haemoglobin and packed cell volume were lower in the SCD children from the south-western province. Haemoglobin A2 level was significantly higher, while haemoglobin F, packed cell volume (PCV), mean corpuscular volume (MCV), and mean corpuscular haemoglobin concentration (MCHC) did not show any significant differences. HbF level did not influence the haematological parameters significantly in the SWP patients. It is concluded that the SCD in Saudi population is heterogeneous clinically and haematologically.