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Thalassemias are group of genetically transmitted autosomal recessive disorder in globin chain production in the haemoglobin (Hb) molecules. beta-thalassemia major is a rare disease caused by complete deficiency of beta-globin chain synthesis .The Frequent blood transfusions and surgical  removal of enlarged spleen are the only options to improve life expectancy and quality of life. Bone marrow transplant is the only definitive cure for haemoglobin diseases such as thalassaemia major. A 7 years old male child with  beta-thalassemia major presented with massive splenomegaly and severe anaemia persisted despite frequent transfusions, thus he was scheduled for elective splenectomy to alleviate anaemia. His preoperative investigation showed marked anaemia, mild liver impairment and moderate pulmonary  hypertension with mitral valve prolapse. Anaesthesia was maintained with balanced anaesthesia using opioids and low dose of volatile anaesthetic agent to avoid cardiovascular depression. Throughout the operation, his vital signs were kept stable. We report successful anesthetic management with attention to hemodynamic changes in a patient with beta thalassemia major.


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