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Biomarkers of splenic function in infants with sickle cell anemia: baseline data from the BABY HUG Trial
Zora R Rogers1, Winfred C Wang, Zhaoyu Luo
1Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX 75390-9063, USA. Zora.Rogers@UTSouthwestern.edu
Insights
Early spleen dysfunction in sickle cell anemia infants is common. Pitted cell counts and Howell-Jolly bodies are reliable biomarkers for assessing spleen function in children.
Area of Science:
- Pediatrics
- Hematology
- Medical Imaging
Background:
- Sickle cell anemia (SCA) frequently leads to spleen dysfunction in early childhood.
- Early identification of spleen function loss is crucial for timely intervention and preventive care.
Purpose of the Study:
- To evaluate spleen function in infants with SCA using liver-spleen scans and novel biomarkers.
- To correlate spleen function with clinical and laboratory findings in young children with SCA.
Main Methods:
- Utilized (99m)Tc sulfur-colloid liver-spleen scans in 193 children aged 8-18 months with SCA.
- Assessed pitted cell counts (PIT) and quantitative Howell-Jolly bodies (HJB) via flow cytometry as splenic biomarkers.
- Correlated scan results with clinical data, including hemoglobin levels, white blood cell counts, and reticulocyte counts.
Main Results:
- Spleen function loss was observed in 86% of infants before 12 months, associated with specific hemoglobin and blood cell count patterns.
- Pitted cell counts and Howell-Jolly bodies showed good correlation with liver-spleen scan results.
- Established new predictive thresholds for normal and absent spleen function using PIT (≤1.2% and ≥4.5%) and HJB (≤55 and ≥665/10(6) RBCs).
Conclusions:
- Early spleen dysfunction is prevalent in SCA infants, underscoring the need for prompt diagnosis and care.
- Both PIT and HJB are validated biomarkers for spleen function in SCA.
- Howell-Jolly bodies offer methodological advantages over pitted cell counts for assessing spleen function.
Abstract:
We evaluated spleen function in 193 children with sickle cell anemia 8 to 18 months of age by (99m)Tc sulfur-colloid liver-spleen scan and correlated results with clinical and laboratory parameters, including 2 splenic biomarkers: pitted cell counts (PIT) and quantitative Howell-Jolly bodies (HJB) enumerated by flow cytometry. Loss of splenic function began before 12 months of age in 86% of infants in association with lower total or fetal hemoglobin and higher white blood cell or reticulocyte counts, reinforcing the need for early diagnosis and diligent preventive care. PIT and HJB correlated well with each other and liver-spleen scan results. Previously described biomarker threshold values did define patients with abnormal splenic function, but our data suggest that normal spleen function is better predicted by PIT of ≤1.2% or HJB ≤55/10(6) red blood cells and absent function by PIT ≥4.5% or HJB ≥665/10(6). HJB is methodologically advantageous compared with PIT, but both are valid biomarkers of splenic function. This trial was registered at www.clinicaltrials.gov as #NCT00006400.