Splenectomy in sickle cell haemoglobinopathies

Abstract

Background: Sickle cell disease is one of the common haemoglobinopathies in the world. Among its various clinical presentations, splenic complications are associated with high morbidity and substantial mortality, the only effective preventive strategy for which is prophylactic splenectomy. The aim of the present study was to observe the safety and effectiveness of splenectomy to prevent frequent requirement of hospitalizations, blood transfusions and splenic complications in patients of sickle cell disease.Methods: The study was carried out in 72 patients of SCD with splenomegaly who underwent splenectomy for some indication in the surgery department of VSSIMSAR, Burla during the period from October 2016 to September 2018. Their preoperative baseline hematological parameters and frequency of transfusion requirement was compared with postoperative status.Results: Of these 72 SCD patients, 49 (68.7%) patients had repeated requirement of blood transfusion and hypersplenism, 22 (30.5%) patients had history of one or more episodes of splenic sequestration crises, one patient had splenic abscess. After splenectomy the mean increase in haemoglobin level, TLC and TPC was respectively 2.83±0.9 gm%, 1.7±0.8 lac/cmm and 2726±1618/cmm. Operative mortality was 0%. None of the patients required any blood transfusion and no major postoperative complications during 6 months follow up period.Conclusions: The morbidity of the patients of SCD in terms of repeated hospitalizations, blood transfusion, living with a huge spleen, accompanying symptoms and its complications can be effectively minimized by the elective splenectomy. With good preoperative preparation and post-operative management, splenectomy in SCD patients is a safe procedure with minimal risk of post-operative complications.

Prevalence of Splenomegaly and Splenic Complications in Adults with Sickle Cell Disease and Its Relation to Fetal Hemoglobin

“…Although the exact mechanism of high HbF in the Indian population is still not understood, it has been well documented that HbF is a very powerful modulator of the clinical and the haematologic features of sickle cell anemia. It has been found that the Saudi-Indian and Senegal haplotypes of HBB-like gene cluster have exceptionally higher HbF levels, hence associated with milder disease and lesser episodes of crisis 32 . In our study, we found that there was a higher level of an average of HbF in patients with splenomegaly (14.90±5.08) compared to those with a normal spleen (12.12±4.12).…”

The Correlation Between Surgical Procedures and Quality of Life Among Sickle Cell Disease Patients: A Perspective Saudi Study

“…These indications can cause pain, which may lower QoL. If these patients undergo a splenectomy, this could lower the frequency of their hospitalization for blood transfusions, provide relief from an enlarged spleen and its symptoms, and prevent recurrent acute splenic sequestration crises and their complications [ 25 ]. However, we could not find an association between QoL and splenectomy.…”

Splenectomy perspective for non-malignant hematological disorders: A cross-sectional study in the Eastern Province of KSA

“…The postoperative hospital stay after a splenectomy procedure can vary, with an average length between 3 and 9 days. 18 , 19 , 20 However, a study has reported that laparoscopic splenectomy for benign hematological disorders is significantly associated with prolonged postoperative hospital stays and greater blood loss. 17 In our study, most patients had a hospital stay period between 2 and 7 days.…”

“…This time period is similar to that in the general population and less than that previously reported in patients with hematological diseases who underwent splenectomy. 18 , 19 , 20…”