Intrathoracic extramedullary haematopoiesis complicated by massive haemothorax in alpha-thalassaemia

Intrathoracic extramedullary haematopoiesis complicated by massive haemothorax in alpha-thalassaemia
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α-地中海贫血胸内髓外造血并发大量血胸

DOI:
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发表时间:
1999
期刊:
影响因子:
10
通讯作者:
Hung
Hung
中科院分区:
医学1区
文献类型:
--
作者:
Kuo;R. Lai;Chien;Jau;Huang;Hung

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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.
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