Outcome following splenectomy in young thalassemic patients aged five to eighteen years. A single centre observational study.

Genç talasemi hastalarında (5-18 yaşları arası) yapılan splenektomi sonrası izlem sonuçları. Tek merkezli gözlemsel bir çalışma

Authors

  • Paul Dibyashree https://orcid.org/0000-0003-1615-5878
  • Arkaprovo Roy https://orcid.org/0000-0002-1025-0594
  • Shuchismita Chakraborty https://orcid.org/0000-0001-6420-9557
  • Alamgir Hossain

Keywords:

Thalassemia; splenectomy; outcome.

Abstract

Excessive destruction of abnormal red blood cells and resultant extra-medullary haematopoiesis in beta thalassemia major leads to splenomegaly, hypersplenism and increased requirement of blood transfusion, which often necessitate splenectomy.

To evaluate the outcome of splenectomy among patients with beta thalassemia major

A retrospective cross-sectional study was conducted on 26 beta thalassemia major patients, in the age group of 5 to18 years, who underwent elective splenectomy in a tertiary hospital between April, 2016 and May, 2018. Their pre-operative, peri-operative and post-operative follow up data were collected from a prospectively maintained database and analysed.

Out of 26 patients, 8 (30.8%) were female and 18 (69.2%) male. Mean age of our study population was 9.3 years. Mean haemoglobin at the time of admission was 5.76 +/- 0.49 g/dl and mean pre-operative blood transfusion requirement was 31.77 +/- 9.34 units/yr. Median length of post-operative hospital stay was 6 days. Immediate post-operative complications included haemorrhage in 1 (3.8%) patient, sepsis in 1 (3.8%), pulmonary complication in 1 (3.8%), wound infection in 2 (7.7%) and death of 2 (7.7%) patients. 19 (73.1%) patients did not encounter any post-operative complication. The mean blood transfusion requirement after splenectomy, as assessed in median follow-up over 26 months, was 13.00+/-7.15 units/yr.

In conclusion, for patients with beta thalassemia major being managed in resource limited set-up, splenectomy can be considered as an effective procedure for reducing the blood transfusion requirement and hence, prevent its associated adverse effects.

ÖZET

Anormal kırmızı kan hücrelerinin aşırı yıkımı ve bunun sonucunda beta talasemi majörde ekstra-medüller hematopoez, splenomegaliye, hipersplenizme ve sıklıkla splenektomi gerektiren kan transfüzyonu gereksiniminin artmasına neden olur.

Amacımız beta talasemi majörlü çocuk hastalarda splenektomi sonuçlarını değerlendirmektir.

Üçüncü basamak bir hastanede Nisan 2016 ve Mayıs 2018 tarihleri arasında elektif splenektomi uygulanan 5-18 yaş grubundaki 26 beta talasemi majör hastası üzerinde retrospektif kesitsel bir çalışma yapılmıştır. Ameliyat sonrası takip verileri ileriye dönük olarak tutulan bir veri tabanından toplandı ve analiz edildi.

26 hastanın 8'i (%30,8) kadın, 18'i (%69,2) erkekti. Çalışma grubumuzun ortalama yaşı 9,3 idi. Başvuru anındaki ortalama hemoglobin 5,76 +/- 0,49 g/dl ve ameliyat öncesi ortalama kan transfüzyon ihtiyacı 31,77 +/- 9,34 ünite/yıl idi. Ameliyat sonrası hastanede kalış süresi ortalama 6 gündü. Postoperatif ani komplikasyonlar 1 (%3,8) hastada kanama, 1 (%3,8) hastada sepsis, 1 (%3,8) hastada pulmoner komplikasyon, 2 (%7,7) hastada yara enfeksiyonu ve 2 (%7,7) hastada ölüm şeklindeydi. 19 (%73,1) hastada postoperatif herhangi bir komplikasyonla karşılaşmadı. 26 aylık medyan takipte, splenektomi sonrası ortalama kan transfüzyonu gereksinimi 13.00+/-7.15 ünite/yıl idi.

Sonuç olarak, sınırlı kaynaklarla tedavi edilen beta talasemi majör hastalarında splenektomi, kan transfüzyonu gereksinimini azaltmak ve dolayısıyla ilişkili olumsuz etkileri önlemek için etkili bir prosedür olarak kabul edilebilir.

Author Biographies

Paul Dibyashree, https://orcid.org/0000-0003-1615-5878

Medical College, Kolkata, India

Arkaprovo Roy, https://orcid.org/0000-0002-1025-0594

Medical College, Kolkata, India

Shuchismita Chakraborty, https://orcid.org/0000-0001-6420-9557

Medical College, Kolkata, India

Alamgir Hossain

Medical College, Kolkata, India

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Published

2023-01-23

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