Frequency and predictors of blood transfusion and splenectomy in pediatric hereditary spherocytosis – a single-center retrospective analysis

Introduction
Hereditary spherocytosis (HS) is the most common hereditary hemolytic anemia among children in Europe. It is characterized by membrane protein defects leading to spherocyte formation and increased hemolysis. Hereditary spherocytosis can present with a wide spectrum of clinical severity, from asymptomatic cases to severe anemia requiring multiple interventions. The aim of the study was to evaluate the frequency and predictors of blood transfusions and splenectomy in children with HS, considering factors such as sex, age at diagnosis, family history, and laboratory parameters.

Material and methods
A retrospective analysis was conducted on 37 pediatric patients with HS treated in 2019–2024 at the Children’s University Hospital in Lublin. Data on sex, age at diagnosis, family history, clinical symptoms, laboratory findings, and treatment were collected. Statistical analysis was performed with a significance level of p < 0.05.

Results
Transfusions were required more frequently in boys (64.7%) than in girls (40%). Splenectomy was performed in 27% of patients, more often in boys. A positive family history was associated with a higher need for transfusions (up to 7 episodes), more frequent anemia, and earlier diagnosis. Low hemoglobin levels (< 8 g/dl) strongly predicted transfusion need. No significant correlation between reticulocyte count and transfusions was observed. Early diagnosis (within the first year of life) was linked to a more severe clinical course and increased splenectomy rate.

Conclusions
Positive family history, low hemoglobin at diagnosis, and early age at presentation appear to be linked with a more severe disease course. Male sex showed a trend toward more severe disease, though this finding should be interpreted with caution. Because of the limited sample size and retrospective nature of the study, the findings should be regarded as descriptive and primarily intended to generate hypotheses. Larger multicenter studies are required to confirm these trends.