Splenectomy in two children with autoimmune lymphoproliferative syndrome and massive splenomegaly

Mia Glerup1, Thomas Thelle, Kirsten Grønbaek

  • 1Department of Pediatrics, Aarhus University Hospital Skejby, Aarhus, Denmark.

Insights

Splenectomy offers a potential treatment for Autoimmune Lymphoproliferative Syndrome (ALPS) in children with enlarged spleens. This study shows splenectomy improved symptoms and well-being in two pediatric ALPS patients.

Area of Science:

  • Pediatric Hematology
  • Immunology
  • Surgical Oncology

Background:

  • Autoimmune Lymphoproliferative Syndrome (ALPS) treatment lacks a clear consensus.
  • Splenectomy is a potential therapeutic option for ALPS, but data in pediatric patients is scarce.

Observation:

  • Two pediatric patients with ALPS presented with significant splenomegaly, anemia, and discomfort.
  • Both patients underwent successful splenectomy with no complications.

Findings:

  • Post-splenectomy, patients experienced marked improvement in cytopenia, daily activities, and overall well-being.
  • The youngest patient exhibited significant catch-up growth following the procedure.

Implications:

  • Splenectomy may be a viable treatment option for carefully selected pediatric ALPS patients with severe splenomegaly.
  • Further research is warranted to establish definitive guidelines for splenectomy in pediatric ALPS management.

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