Goh Nicole S L, McDonald Christine E, MacGregor Duncan P, Pretto Jeffrey J, Brodie Graeme N
Royal Brompton Hospital, London, United Kingdom.
Respirology. 2003 Mar;8(1):91-4. doi: 10.1046/j.1440-1843.2003.00427.x.
Langerhans cell histiocytosis (LCH) is a rare disorder which frequently involves the lungs of affected adults. Recent evidence suggests it is a clonal neoplastic disorder. Prognosis in this disease is variable, but in its multisystem form or when associated with progressive respiratory dysfunction, prognosis is poor. Recent case reports and a phase II trial of the antimonocyte drug 2-chlorodeoxy-adenosine (2CDA) have described success in treating LCH. We used 2CDA to treat a young Australian man with LCH involving lungs and bone. A complete symptomatic remission was achieved with no evidence of recurrence some 5 years after completion of chemotherapy.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见的疾病，在受影响的成年人中常累及肺部。最近的证据表明它是一种克隆性肿瘤性疾病。这种疾病的预后各不相同，但在其多系统形式或与进行性呼吸功能障碍相关时，预后较差。最近的病例报告和一项关于抗单核细胞药物2-氯脱氧腺苷（2CDA）的II期试验描述了治疗LCH的成功案例。我们使用2CDA治疗一名患有肺部和骨骼受累的LCH的年轻澳大利亚男子。化疗完成约5年后，实现了完全症状缓解，且无复发迹象。
