Splenectomy in Children with Beta Thalassemia Major and Sickle Cell Disease: A 4-Year Experience from Iraq

Background: Splenectomy is indicated as a therapeutic option in different types of hereditary blood disorders. Objective: The aim of this study is to determine the outcome of splenectomy in children with beta-thalassemia major (BTM) and sickle cell disease (SCD). Materials and Methods: A retrospecti...

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Main Authors: Sadik H. Kadhem, Basim A. Al Hijaj, Haithem H. Ali, Rawshan Zuhair Jaber
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2025-01-01
Series:Medical Journal of Babylon
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Online Access:https://doi.org/10.4103/MJBL.MJBL_1377_23
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author Sadik H. Kadhem
Basim A. Al Hijaj
Haithem H. Ali
Rawshan Zuhair Jaber
author_facet Sadik H. Kadhem
Basim A. Al Hijaj
Haithem H. Ali
Rawshan Zuhair Jaber
author_sort Sadik H. Kadhem
collection DOAJ
description Background: Splenectomy is indicated as a therapeutic option in different types of hereditary blood disorders. Objective: The aim of this study is to determine the outcome of splenectomy in children with beta-thalassemia major (BTM) and sickle cell disease (SCD). Materials and Methods: A retrospective study was conducted in Basrah-Iraq on children with BTM and SCD who had undergone open splenectomy in the period between January 2016 and December 2019 at our institution. Results: The study included 87 children (49 males and 38 females). 59.8% with SCD and 35 (40.2%) with BTM. The mean age was 8.46 range (3–15) years. The mean weight was 23.51 range (11–45) kg. In SCD, the major indication (71.1%) was acute splenic sequestration (ASS). Postsplenectomy outcome in BTM included improvement in the median hemoglobin (Hb) level by 2.1 g/dL, reduction in the median packed red blood cells (PRBC) transfusion frequency by 19/year, and reduction in the median PRBC requirement by 190 mL/kg/year. In SCD, improvement in the median Hb level by 1.55 g/dL, reduction in the median PRBC transfusion frequency by 4.5/year, and reduction in the median PRBC requirement by 45 mL/kg/year. In addition, splenectomy eliminated life-threatening ASS crisis. Postoperative complications were observed in 6.9% of patients. Conclusions: SCD was the main hematological condition requiring splenectomy. ASS was the main indication for splenectomy in SCD. Splenectomy has proved beneficial in the management of SCD and BTM patients. Furthermore, it is now recognized as a safe surgery with a low complication rate.
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spelling doaj-art-1a7301e4c2e04db78f0360e2907102ef2025-08-20T01:51:41ZengWolters Kluwer Medknow PublicationsMedical Journal of Babylon1812-156X2312-67602025-01-0122120120510.4103/MJBL.MJBL_1377_23Splenectomy in Children with Beta Thalassemia Major and Sickle Cell Disease: A 4-Year Experience from IraqSadik H. KadhemBasim A. Al HijajHaithem H. AliRawshan Zuhair JaberBackground: Splenectomy is indicated as a therapeutic option in different types of hereditary blood disorders. Objective: The aim of this study is to determine the outcome of splenectomy in children with beta-thalassemia major (BTM) and sickle cell disease (SCD). Materials and Methods: A retrospective study was conducted in Basrah-Iraq on children with BTM and SCD who had undergone open splenectomy in the period between January 2016 and December 2019 at our institution. Results: The study included 87 children (49 males and 38 females). 59.8% with SCD and 35 (40.2%) with BTM. The mean age was 8.46 range (3–15) years. The mean weight was 23.51 range (11–45) kg. In SCD, the major indication (71.1%) was acute splenic sequestration (ASS). Postsplenectomy outcome in BTM included improvement in the median hemoglobin (Hb) level by 2.1 g/dL, reduction in the median packed red blood cells (PRBC) transfusion frequency by 19/year, and reduction in the median PRBC requirement by 190 mL/kg/year. In SCD, improvement in the median Hb level by 1.55 g/dL, reduction in the median PRBC transfusion frequency by 4.5/year, and reduction in the median PRBC requirement by 45 mL/kg/year. In addition, splenectomy eliminated life-threatening ASS crisis. Postoperative complications were observed in 6.9% of patients. Conclusions: SCD was the main hematological condition requiring splenectomy. ASS was the main indication for splenectomy in SCD. Splenectomy has proved beneficial in the management of SCD and BTM patients. Furthermore, it is now recognized as a safe surgery with a low complication rate.https://doi.org/10.4103/MJBL.MJBL_1377_23childrensickle cell diseasesplenectomysplenic sequestrationthalassemiatransfusion
spellingShingle Sadik H. Kadhem
Basim A. Al Hijaj
Haithem H. Ali
Rawshan Zuhair Jaber
Splenectomy in Children with Beta Thalassemia Major and Sickle Cell Disease: A 4-Year Experience from Iraq
Medical Journal of Babylon
children
sickle cell disease
splenectomy
splenic sequestration
thalassemia
transfusion
title Splenectomy in Children with Beta Thalassemia Major and Sickle Cell Disease: A 4-Year Experience from Iraq
title_full Splenectomy in Children with Beta Thalassemia Major and Sickle Cell Disease: A 4-Year Experience from Iraq
title_fullStr Splenectomy in Children with Beta Thalassemia Major and Sickle Cell Disease: A 4-Year Experience from Iraq
title_full_unstemmed Splenectomy in Children with Beta Thalassemia Major and Sickle Cell Disease: A 4-Year Experience from Iraq
title_short Splenectomy in Children with Beta Thalassemia Major and Sickle Cell Disease: A 4-Year Experience from Iraq
title_sort splenectomy in children with beta thalassemia major and sickle cell disease a 4 year experience from iraq
topic children
sickle cell disease
splenectomy
splenic sequestration
thalassemia
transfusion
url https://doi.org/10.4103/MJBL.MJBL_1377_23
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AT haithemhali splenectomyinchildrenwithbetathalassemiamajorandsicklecelldiseasea4yearexperiencefromiraq
AT rawshanzuhairjaber splenectomyinchildrenwithbetathalassemiamajorandsicklecelldiseasea4yearexperiencefromiraq