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Thorax 1999;54:466-468 ( May )

Case report

Intrathoracic extramedullary haematopoiesis complicated by massive haemothorax in alpha-thalassaemia Kuo-An Chu,a Ruay-Sheng Lai,a d Chien-Hong Lee,e Jau-Yeong Lu,a d Huang-Chou Chang,c Hung-Ting Chiangb d

a Division of Chest Medicine, b Department of Internal Medicine, c Division of Thoracic Surgery, d Veterans General Hospital-Kaohsiung, Kaohsiung, Taiwan, Republic of China Faculty of Medicine, National Yang-Ming University, Taiwan, Republic of China, e School of Medical Technology, Chung Gung University, Taiwan, Republic of China

Correspondence to: Dr R-S Lai, Division of Chest Medicine, Veterans General Hospital-Kaohsiung 386, Ta-Chung 1st Road, Kaohsiung, Taiwan.

Received 13 February 1997; Returned to authors 13 May 1997; Revised version received 15 July 1997; Accepted for publication 3 December 1997

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 alpha alpha CS/alpha alpha T. 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.


Keywords: extramedullary haematopoiesis; haemothorax; thalassaemia

© 1999 by Thorax


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