Intrathoracic extramedullary haematopoiesis complicated by massive haemothorax in alpha-thalassaemia
- Kuo-An Chua,
- Ruay-Sheng Laia,d,
- Chien-Hong Leee,
- Jau-Yeong Lua,d,
- Huang-Chou Changc,
- Hung-Ting Chiangb,d
- aDivision of Chest Medicine, bDepartment of Internal Medicine, cDivision of Thoracic Surgery, dVeterans General Hospital-Kaohsiung, Kaohsiung, Taiwan, Republic of China Faculty of Medicine, National Yang-Ming University, Taiwan, Republic of China, eSchool of Medical Technology, Chung Gung University, Taiwan, Republic of China
- Dr R-S Lai, Division of Chest Medicine, Veterans General Hospital-Kaohsiung 386, Ta-Chung 1st Road, Kaohsiung, Taiwan.
- Received 13 February 1997
- Revision requested 13 May 1997
- Revised 15 July 1997
- Accepted 3 December 1997
Abstract
Intrathoracic extramedullary haematopoiesis (EMH) is a rare entity that is usually asymptomatic. A 44 year old man with alpha-thalassaemia is described who developed dyspnoea and massive left sided haemothorax. The haemoglobin disorder was established by Hgb H staining and haemoglobin electrophoretic studies. The DNA analysis revealed it to be a case of double heterozygous terminal codon mutation with the genotype ααCS/ααT. Computed tomographic scanning and magnetic resonance imaging of the thorax showed multiple paravertebral masses which were found by thoracoscopic biopsy to be extramedullary haematopoiesis. Although no additional sclerosing pleurodesis or low dose radiation therapy was given, the lung expanded well and there has been no recurrence of haemothorax to date.
