IMRT/IGRT Helical Tomotherapy: A Successful Treatment of Lung Parenchyma Compression Due to Extramedullary Haematopoiesis in Β-thalassaemia – a Case Report

Abstract

Thalassaemia is a chronic haemolytic anaemia that is endemic in the Mediterranean Basin. Extramedullary haematopoiesis (EMH) is a natural compensatory reaction involving several organs or tissues. This report outlines a case of dyspnoea due to bilateral dorsal paravertebral EMH, which was treated successfully with helical tomotherapy, a technique that combines intensity-modulate radiation therapy with image-guided radiation therapy. By the end of the first week of treatment, an increase in the haemoglobin value (up to 7.8 g/dL) and a remarkable reduction of dyspnoea were obtained, with haemoglobin values maintained at 7.8–7.3 g/dL without further blood transfusions. At 1-year follow-up, the patient was totally asymptomatic, with a complete resolution of dyspnoea and asthenia. The therapeutic approach to EMH remains controversial because there are no pre-established protocols, with current treatments including serial blood transfusions, hydroxyurea, radiation therapy, and surgical decompression. This clinical case description reports how helical tomotherapy may be used as a valid and effective treatment for compressive atelectasis due to EMH. In fact, radiation therapy improved the general clinical condition and self-reported symptoms of the patient and reduced the size of EMH masses visible in the chest CT scan.