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ANZSCTS ASM 2024
ANZSCTS ASM 2024

Acute Spontaenous Massive Haemothorax in a Jehovah’s Witness with beta-thalassemia and thoracic extramedullary haematopoiesis: a management conundrum

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Presentation Description

Institution: Fiona Stanley Hospital - Western Australia, Australia

A 37 year-old male presented with acute atraumatic left sided chest pain and dyspnoea. The patient had a medical history significant for beta-thalassemia with baseline haemoglobin concentration 80-90 g/L, and thoracic extramedullary haematopoiesis. He identified as a Jehovah’s Witness. Initial x-ray imaging revealed a rapidly progressive large left pleural collection. There was acute anaemia with Haemoglobin drop from 80 g/L at admission, to 53 g/L within 36hrs of presentation. The patient was tachypnoic and tachycardic, without hypoxia or hypotension. Subsequent CT angiography of the chest revealed large left-sided pleural effusion compatible with haemothorax, passive atelectasis and near complete collapse of the left lower lung, and posterior mediastinal and paraspinal extramedullary haematopoiesis. There was no active bleeding identified at the time of the scan and therefore no suitable target for endovascular embolization. Multidisciplinary management input was sought from cardiothoracic surgery, haematology, interventional radiology, and, and radiation oncology. In accordance with his religious beliefs, the patient declined administration of resuscitative blood products. A decision was undertaken to stabilize the patient medically prior to any invasive thoracic intervention, to reduce the risk of iatrogenic bleeding without the option of subsequent blood transfusion. The patient was admitted to hospital and received tranexamic acid, high dose Erythropoietin, iron infusion, and B12 and folate supplementation. After 10 days, the haemoglobin concentration improved to 88 g/L. A left intercostal chest drain was inserted by a cardiothoracic surgeon under local anaesthetic. Four litres of dark blood-stained fluid was drained and there was partial re-expansion of the left lung. The chest drain was removed after 48hrs. The patient subsequently underwent targeted radiotherapy of the extramedullary haematopoietic tissue, to reduce the risk of future recurrent spontaneous bleeding.

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