Long‐term hematologic and clinical outcomes of splenectomy in children with hereditary spherocytosis and sickle cell disease

Abstract

Background: Total splenectomy (TS) and partial splenectomy (PS) are used for children with congenital hemolytic anemia (CHA), although the long-term outcomes of these procedures are poorly defined. This report describes long-term outcomes of children with CHA requiring TS or PS. Procedure:We collected data from children ages 2-17 with hereditary spherocytosis (HS) or sickle cell disease (SCD) requiring TS or PS from 1996 to 2016 from 14 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a prospective, observational patient registry. We summarized hematologic outcomes, clinical outcomes, and adverse events to 5 years after surgery. Hematologic outcomes were compared using mixed effects modeling.Results: Over the study period, 110 children with HS and 97 children with SCD underwent TS or PS. From preoperatively compared to postoperatively, children with HS increased their mean hemoglobin level by 3.4 g/dL, decreased their mean reticulocyte percentage by 6.7%, and decreased their mean bilirubin by 2.4mg/dL. Hematologic improvements and improved clinical outcomes were sustained over 5 years of follow-up. For children with SCD, there was no change in hemoglobin after PS or TS following surgery, although all clinical outcomes were improved. Over 5 years, there was one child with HS and 5 children with SCD who developed post-splenectomy sepsis.Conclusions: For children with HS, there are excellent long-term hematologic and clinical outcomes following either PS or TS. Although hemoglobin levels do not change after TS or PS in SCD, the long-term clinical outcomes for children with SCD are favorable.

Morbidity and mortality of sickle cell disease patients is unaffected by splenectomy: evidence from three decades of follow-up in a high-income setting

“…Based on our records, antibiotic prophylaxis was generally discontinued either after the age of 14 years or at one year after splenectomy, associated with patient and care-givers education. These agrees with results from four different studies, which analyzed smaller SCD populations and for a shorter period of time when compared to our study 6,7,15,16 . Similar results were also reported in two different studies from low-income countries with a follow-up of 18 months and three years after splenectomy 10,14,17 .…”

“…Our findings are concordant with results from four different studies which analyzed smaller SCD populations and for a shorter period of time compared to our study. 6 , 7 , 15 , 16 Similar results were also reported for two different studies from low-income countries with a follow-up of 18 months and 3 years after splenectomy. 10 , 14 , 17 In our cohort, the absence of a significant difference in fatal infectious events between splenectomized and non-splenectomized patients with SCD might be related to a combination of vaccination, patient education and the intensive follow-up program conducted in comprehensive care centers for hemoglobinopathies by expert medical staff.…”

Current Status of Molecular Diagnosis of Hereditary Hemolytic Anemia in Korea

“…Patients with CDA-II, hexokinase deficiency, G6PD type 1 deficiency, and PKD, who require transfusions, may be candidates for splenectomy. 106 Grace et al 48 showed that individuals with two non-missense mutations in PKD responded poorly to splenectomy, as observed in Amish patients. Therefore, these factors should be considered when planning treatment.…”

Partial splenectomy in children: Long-term reoperative outcomes☆

“…Given its relative novelty compared to total splenectomy for the management of hematologic diseases in children, there is less known about partial splenectomy's short and long-term success in controlling hematologic disease sufficiently to prevent completion splenectomy. To date, studies have demonstrated that the effects of PS postoperatively can be variable and are dependent on the primary hematologic condition; in HS, PS has shown both improved postoperative hematologic parameters and clinical symptoms over 1-and 5-years [2,3,11,12]. This response, however, may not be as robust as cases where a total splenectomy is performed and suggests that hematologic improvement is correlated with extent of splenectomy.…”

“…This response, however, may not be as robust as cases where a total splenectomy is performed and suggests that hematologic improvement is correlated with extent of splenectomy. This may render partial splenectomy patients at risk for disease recurrence and in need of a secondary splenectomy procedure otherwise not observed in those who undergo a total splenectomy at onset [2,3,5,[11][12][13].…”