Improved Splenic Function After Hematopoietic Stem Cell Transplant for Sickle Cell Disease

Area of Science:

• Hematology
• Pediatric Medicine
• Transplant Science

Background:

• Sickle cell disease (SCD) frequently causes splenic dysfunction.
• Hematopoietic stem cell transplant (HSCT) offers a cure for SCD, but its long-term impact on spleen function requires further investigation.

Purpose of the Study:

• To evaluate the long-term effects of HSCT on splenic function in pediatric patients with SCD.
• To identify risk factors associated with poor splenic function after HSCT for SCD.

Main Methods:

• Retrospective cohort study of pediatric SCD patients undergoing HSCT at two centers.
• Blind review of technetium-99m liver-spleen (LS) scans to assess splenic uptake (absent, decreased, normal).

Main Results:

• Post-HSCT, 75% of patients showed decreased splenic uptake and 9% absent uptake, a significant improvement from pre-HSCT rates (37% had uptake).
• Older age at HSCT (median 16.6 years) was associated with absent splenic uptake compared to those with present uptake (median 8.0 years).
• Extensive chronic graft-versus-host disease (GVHD) was more prevalent in patients with absent splenic uptake (40%) versus present uptake (6%).

Conclusions:

• HSCT substantially enhances splenic function in the majority of pediatric SCD patients.
• Advanced age at the time of HSCT and extensive chronic GVHD are identified as risk factors for suboptimal splenic function post-transplant.