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Leucocyte counts in children with sickle cell anaemia usefulness of stable state values during infections
1First Consultants Medical Centre, Ltd., Obalende, Lagos.
Insights
Children with sickle cell anemia have higher white blood cell counts, including neutrophils, than healthy children. These elevated counts mean current infection diagnosis criteria may not apply to sickle cell patients.
Area of Science:
- Hematology
- Pediatrics
- Immunology
Background:
- Sickle cell anemia (SCA) is a genetic blood disorder characterized by abnormal hemoglobin.
- Elevated white blood cell (WBC) counts are common in SCA, but their implications for infection diagnosis are unclear.
Purpose of the Study:
- To establish steady-state cell counts in children with SCA.
- To compare these counts with those of healthy children (AA genotype).
- To evaluate the diagnostic criteria for bacterial infections in SCA patients.
Main Methods:
- Compared total white blood cell (WBC) and neutrophil counts in 200 steady-state SCA children and 60 age/sex-matched controls.
- Analyzed total white blood cell (T-WBC), total polymorphonuclear neutrophil (T-PMN), segmented neutrophil (T-S), and non-segmented neutrophil (T-NS) counts.
- Assessed the percentage of polymorphonuclear neutrophils and its correlation with age.
Main Results:
- Steady-state SCA patients showed significantly higher mean T-WBC, T-PMN, T-S, and T-NS counts compared to controls (P < 0.001 for most).
- SCA patients had a lower percentage of polymorphonuclear neutrophils (34%) than expected, which did not significantly change with age (P > 0.05).
- The elevated WBC and neutrophil counts in SCA patients differ from typical responses seen in non-SCA individuals.
Conclusions:
- Established reference ranges for steady-state cell counts in pediatric SCA.
- The elevated counts suggest existing diagnostic criteria for bacterial infections are inadequate for SCA patients.
- New diagnostic criteria are needed for identifying bacterial infections in children with sickle cell anemia.
Abstract:
The total white blood cell count and total and differential neutrophil counts were compared in two hundred steady state sickle cell anaemia children and sixty age and sex matched AA genotype controls to establish steady state cell counts. Steady state patients were found to have significantly higher mean total white blood cell counts (T-WBC--12.4 +/- 3.0 x 10(9/1)), mean total polymorphonuclear neutrophil counts (T-PMN--4.2 +/- 1.2 x 10(9/1)), mean total segmented (T-S--2.7 +/- 0.9 x 10(9/1)), and mean total non-segmented neutrophil counts (T-NS--1.5 +/- 0.5 x 10(9/1)), when compared with their M genotype controls; T-WBC (5.2 +/- 1.9 x 10(9/1)) (P < 0.001), T-PMN (2.2 +/- 8.0 x 10(9/1)) (P < 0.001), T-S (2.2 +/- 0.9 x 10(9/1)) (P < 0.01), and T-NS (0.02 +/- 0.04 x 10(9/1)) (P < 0.001). Contrary to an expected neutrophilia, steady state patients had a low percentage polymorphonuclear neutrophil count (34%) which did not change significantly with increasing age (P > 0.05). The raised counts in steady state patients suggest that previously established criteria for the diagnosis of bacterial infections cannot be applied to sickle cell patients, and thus new criteria must be generated.