Anesthetic management of a complex spine surgery in a thalassemia patient: a path less travelled!

Spinal extramedullary hematopoiesis (EMH) a rare complication of Hb E/β-thalassemia can result in compressive myelopathy, posing serious neurological risks, particularly in the thoracic spine. We present the case of a 32-year-old male with Hb E/β-thalassaemia who developed spastic paraplegia due to compressive myelopathy from thoracic epidural EMH refractory to radiotherapy. The patient had severe hypochromic microcytic anemia (Hb 7.3 g/dL), elevated serum iron, and significant spinal canal narrowing from D4-D10 on MRI. The patient underwent surgical decompression under general anesthesia with special considerations, including careful airway management, prone positioning, and restrictive transfusion protocols to avoid increased blood viscosity and exaggerated hemolysis. This case highlights the multifaceted challenges of managing thoracic EMH in Hb E/β-thalassaemia, including ineffective erythropoiesis, hypercoagulability, and anemia. The use of tranexamic acid plays a key role in reducing the amount of intraoperative bleeding and mitigating the risk of hyperfibrinolysis in a prothrombotic condition. The Individualized anesthetic and surgical strategies were crucial for significant neurological recovery and improved quality of life of the patient.