Hematologic outcomes after total splenectomy and partial splenectomy for congenital hemolytic anemia

Abstract

Purpose: The purpose of this study was to define the hematologic response to total splenectomy (TS) or partial splenectomy (PS) in children with hereditary spherocytosis (HS) or sickle cell disease (SCD).

Methods: The Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium registry collected hematologic outcomes of children with CHA undergoing TS or PS to 1 year after surgery. Using random effects mixed modeling, we evaluated the association of operative type with change in hemoglobin, reticulocyte counts, and bilirubin. We also compared laparoscopic to open splenectomy.

Results: The analysis included 130 children, with 62.3% (n=81) undergoing TS. For children with HS, all hematologic measures improved after TS, including a 4.1g/dl increase in hemoglobin. Hematologic parameters also improved after PS, although the response was less robust (hemoglobin increase 2.4 g/dl, p<0.001). For children with SCD, there was no change in hemoglobin. Laparoscopy was not associated with differences in hematologic outcomes compared to open. TS and laparoscopy were associated with shorter length of stay.

Conclusion: Children with HS have an excellent hematologic response after TS or PS, although the hematologic response is more robust following TS. Children with SCD have smaller changes in their hematologic parameters. These data offer guidance to families and clinicians considering TS or PS.

Keywords: Congenital hemolytic anemia; Hematologic outcomes; Splenectomy; Surgical technique.

Fig. 1

Unadjusted hematologic outcomes after partial or total splenectomy in children with hereditary spherocytosis. Data represent hemoglobin (A), reticulocyte count (B), and serum bilirubin (C), at baseline, 4 weeks, 24 weeks, and 52 weeks. Circles represent mean and error bars represent standard error. Because of limited data points available at 52 weeks in children with HS undergoing total splenectomy, data are not displayed.

Fig. 2

Unadjusted hematologic outcomes after partial or total splenectomy in children with sickle cell disease. Data represent hemoglobin (A), reticulocyte count (B), and serum bilirubin (C), at baseline, 4 weeks, 24 weeks, and 52 weeks. Circles represent mean and error bars represent standard error.

Fig. 3

Postsplenectomy changes in hematologic parameters for children with congenital hemolytic anemias. Results represent differences in postoperative hematologic values at 4, 24, and 52 weeks (versus baseline), laparoscopic (versus open) splenectomy, and partial (versus total) splenectomy as indicated. Results produced from random-effects mixed modeling including the following covariates: gender, race/ethnicity, laparoscopic vs. open technique, partial vs. total splenectomy. Hereditary spherocytosis and sickle cell disease were analyzed separately. Blue squares represent point estimates and black lines represent 95% confidence intervals. Results are additive such that estimating the change in postoperative hemoglobin after laparoscopic partial splenectomy, the point estimates for postoperative, laparoscopy, and partial splenectomy would need to be added. For example, the postoperative hemoglobin increase in HS is estimated at 4.1 g/dl in open, total splenectomy. This estimate is decreased by 1.7 g/dl in partial splenectomy compared to total splenectomy (estimate displayed) indicating that the expected postoperative hemoglobin in partial splenectomy would only be 2.4 g/dl (equal to 4.1–1.7; not displayed).