Partial splenic embolization in children with hereditary spherocytosis

Area of Science:

• Pediatric Hematology
• Interventional Radiology
• Genetics and Genetic Diseases

Background:

• Hereditary spherocytosis (HS) management often involves splenectomy, increasing risks of infection and thrombosis.
• Partial splenectomy or embolization (PSE) offers a potential alternative to preserve splenic function and mitigate complications.
• Investigating PSE benefits in pediatric HS is crucial for optimizing treatment strategies.

Purpose of the Study:

• To evaluate the efficacy and safety of partial splenic embolization (PSE) in children diagnosed with moderate to severe hereditary spherocytosis (HS).

Main Methods:

• Eight children (median age 8 years) with moderate to severe HS underwent PSE via retrograde transfemoral access.
• Pre-procedure complications included gallstones (6/8) and aplastic crises (4/8); one patient required transfusions.
• The study monitored for acute side effects during and after the PSE procedure.

Main Results:

• No acute adverse events were observed during or post-PSE.
• Median hemoglobin levels significantly increased from 7.5–11.65 g/dL to 8.4–13.35 g/dL (P = 0.012).
• Median splenic index significantly decreased from 9.7–19.0 cm²/m² to 4.4–15.65 cm²/m² (P = 0.012).

Conclusions:

• Partial splenic embolization (PSE) is a safe and effective therapeutic option for pediatric patients with moderate to severe hereditary spherocytosis (HS).
• PSE demonstrates feasibility in managing HS, improving hematological parameters and reducing spleen size.
• This interventional radiology technique offers a viable alternative to splenectomy, preserving essential immune function.