Splenectomy in Patients with Beta Thalassaemia Major

Authors

  • Tazeen Anwar Thalassaemia Centre, Pakistan Institute of Medical Sciences, Islamabad
  • Gul-e-Najaf Thalassaemia Centre, Pakistan Institute of Medical Sciences, Islamabad
  • Bilal Ahmed Department of Paediatrics , Islamic International Medical College & Railway Hospital, Rawalpindi
  • Faheem Ahmad Subhani Department of Paediatrics , Islamic International Medical College & Railway Hospital, Rawalpindi

Keywords:

Splenectomy, Beta Thalassaemia Major, blood transfusion rate, hypersplenism

Abstract

Objective: To evaluate the effect of splenectomy, in patients with beta thalassaemia major, on blood transfusion rates and blood counts  

Methods: In this prospective descriptive study patients with beta thalassaemia major, who underwent splenectomy were included . Inclusion criteria was thalasseamics  with signs of growth retardation , poor health, leucopenia, thrombocytopenia, increased transfusion demand (more than 250 ml/kg/year or more than four transfusions per month) or respiratory embarrassment (mechanical discomfort) from massive splenomegaly even with lesser transfusion requirements  were included.  The transfusion requirement, pre- and post- splenectomy, was expressed as the volume of packed red cells used for transfusion in one year divided by the weight of the patient in mid year and expressed as ml/kg/year. Hypersplenism was established in patients having splenomegaly with accompanying anaemia, leucopenia, thrombocytopenia  or any combination of these with hypercellular bone marrow .

Results: Majority were male (60.65%).Average hospital stay was six days. Pre-splenectmy, majority of patients had transfusion requirements more than 350 ml/kg/l. Blood trnasfusion requirements revealed a significant fall( less  than 250 ml/kg/l) post splenectomy.One year follow up showed significant fall in transfusion rate and significant increase in haemoglobin . In  23.94%  patients splenectomy was performed at an age less than ten years.

Conclusion : Splenectomy has a positive outcome on blood transfusion  rates and haemoglobin  levels in patients with beta thalassaemia major

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Published

2022-02-13

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Original Article