Large Bilateral Intrathoracic Extramedullary Hematopoiesis (EMH) in a Patient With Thalassaemia Intermedia With Hemothorax and Complete Atelectasis of the Left Lower Lob After Trauma– A Case Report

Background Introduction: Extramedullary Hematopoiesis (EMH) which is defined as an ectopic blood cell production commonly triggered by hematologic disorders like Thalassaemia and myelofibrosis. It is generally rare. Apart from the common sites of extramedullary hematopoiesis, intrathoracic sites have also been documented. Due to the hypervascularization of such sites, a thoracic trauma can lead to massive hemorrhage. We therefore report a case of intrathoracic EMH, presenting with a massive hemothorax on the left side requiring surgery.

Methods & Materials Case presentation: A 54-year-old woman with known Thalassaemia intermedia and large bilateral intrathoracic EMH (9 x 8 cm left and 11 x 9 cm right) first presented with pain in the right knee after falling from a bicycle. A fracture was ruled out in Xray. The knee was immobilized and a prophylaxis against thrombosis with Xarelto was prescribed. 4 weeks later, she presented with left thoracic pain and a computed tomogram of the thorax was done with the diagnosis of massive hemothorax and complete atelectasis of the left lower lob. We decided to operate. Preoperatively, an ultrasound-guided microcoil embolization of the intrathoracic arteries (Level 9-11 left and 10-11 right) was performed. We conducted an open evacuation of the hemothorax and resection of the EMH on the left side due to necrosis [1].

Results After successful resection of the EMH on the left side and evacuation of the hemothorax, the follow up scans showed a complete dilated left lower lob. No major events occured after the operation

Conclusion Careful examination of patients with known intrathoracic EMH should be taken after trauma, as this could lead to massive hemothorax and consequent pulmonary complication like atelectasis and pneumonia.

• Literatur

• 1 Karass M, 1 Linder K, 1 Agarwal A. 1 et al. A 54-year-old Woman with Myelofibrosis and Massive Hemothorax Due to Primary Extramedullary Hematopoiesis of the Pleura. Cureus 2018; 10 (12) e3675 DOI: 10.7759/cureus.3675.

Publication History

Article published online:
13 August 2024