CRA2 INTRATHORACIC EXTRAMEDULLARY HAEMATOPOIESIS PRESENTING AS INTRATHORACIC MASSES – A CASE REPORT AND LITERATURE REVIEW

Jiun Hang Lee1, Larry Ellee Nyanti1 2, Meryl Grace Lansing1 2, Subramaniam Ponnuvelu1, Shan Min Lo1, Nai- Chien Huan1, Hema Yamini Ramarmuty1, Kunji Kannan Sivaraman Kannan1
1 Department of Respiratory Medicine, Hospital Queen Elizabeth, Kota Kinabalu, Sabah, Malaysia.
2 Medical Department, Faculty of Medicine and Health Sciences, University Malaysia Sabah, Kota Kinabalu, Malaysia.

Introduction:

Extramedullary haematopoiesis (EMH) is an abnormal compensatory mechanism due to ineffective haematopoiesis secondary from underlying haematological disorder. Intrathoracic presentation of EMH could signify poor prognosis and severe haemoglobinopathy. 

Case Description:

We describe a case of a 38-year-old gentleman with underlying beta-thalassemia who presented to us with left pleural effusion and rib-based opacities on chest radiograph. Pleuroscopy revealed multiple lobulated masses along the surface of the ribs which are soft in consistency and appeared very vascularized underneath. Decision was made to not proceed with biopsy of the masses. Subsequent computed tomography (CT) of the thorax revealed soft tissue masses with heterogeneities from the ribs. We conducted a brief literature review to compare the presentations, radiological features, management and outcome of intrathoracic EMH

Discussion:

Upfront computed tomography (CT) of thorax or MRI should be done to differentiate intrathoracic EMH from other differential diagnosis such as malignancy. Location of the intrathoracic EMH could determine next-step of management from watchful waiting to excisional biopsy. Treating underlying blood disorder is the main treatment. However, for masses that are found over paraspinal region or posterior mediastinum, biopsy or surgical removal are commonly performed to confirm and eliminate other differential diagnosis, including neurogenic tumors citing the risk of bleeding from the mass and spinal cord compression. 

Conclusion:

Clinicians should be mindful of the potential for EMH when evaluating patients with underlying hematological disorder presenting with intrathoracic masses.