Partial Splenectomy as a Definitive or Interim Solution for Young Children with Transfusion-dependent Hemolytic Anemia: Long-term Results from a 15-Year Cohort.

OBJECTIVES

To evaluate the long-term efficacy and safety of partial splenectomy (PS) as a definitive or interim spleen-preserving strategy in young children with transfusion-dependent congenital hemolytic anemia (HA).

METHODS

Patients with congenital HA who required frequent blood transfusions and underwent PS at the present center between 2010 and 2025 were included. The hemoglobin (Hb) level, reticulocyte count, and the need for transfusions (if any) during the follow-up period were noted. Ultrasonography of the splenic remnant was obtained at baseline, 1 mo, and 18 mo following the PS.

RESULTS

Fourteen children (median age: 23 mo; range: 7-40) underwent PS by laparotomy (n = 12) or laparoscopy (n = 2). Median follow-up after PS was 9 y (IQR: 4.8-10.5). Following PS, 8/14 (57%) patients became transfusion-free, with a median Hb of 10.3 g/dL at last follow-up. Five (36%) patients continued to require occasional transfusions and subsequently underwent total splenectomy after a median of 3 y; All of them became transfusion-free thereafter. No episode of mortality, sepsis, or thrombosis occurred.

CONCLUSIONS

PS may serve as a definitive procedure in selected young children with congenital hemolytic anemia and as a bridge to later total splenectomy in others. This approach may be particularly relevant in resource-limited settings, where blood availability, transfusion safety, and the risk of transfusion-transmitted infections are concerning.