Mayou S C, Chu A C, Munro D D, Plowman N
Department of Dermatology, St Bartholomew's Hospital, London, UK.
Clin Exp Dermatol. 1991 Jul;16(4):292-4. doi: 10.1111/j.1365-2230.1991.tb00379.x.
We describe an adult with progressive LCH who received oral etoposide as primary treatment. The response is documented and the strategical implications of this drug in Langerhans-cell histiocytosis discussed. Langerhans-cell histiocytosis (LCH) is the term recognized since 1987 for the group of diseases previously designated histiocytosis X. It is not now regarded as a malignant neoplastic process and there is some evidence to suggest that it is due to abnormal immunity, but cytotoxic drugs and steroids are still the mainstay of systemic treatment. Etoposide (VP16) is a semisynthetic epipodophyllotoxin derivative effective in the treatment of malignancies of the monocyte-macrophage lineage and used in resistant or relapsed childhood LCH. There are no previous reports of its use as firstline monotherapy in adults with LCH.
我们描述了一名接受口服依托泊苷作为主要治疗的成年进展性朗格汉斯细胞组织细胞增多症（LCH）患者。记录了治疗反应，并讨论了该药物在朗格汉斯细胞组织细胞增多症中的策略意义。朗格汉斯细胞组织细胞增多症（LCH）是自1987年以来用于先前称为组织细胞增多症X的一组疾病的术语。它现在不被视为恶性肿瘤过程，并且有一些证据表明它是由于免疫异常引起的，但细胞毒性药物和类固醇仍然是全身治疗的主要手段。依托泊苷（VP16）是一种半合成的鬼臼毒素衍生物，对单核细胞 - 巨噬细胞谱系的恶性肿瘤有效，用于治疗耐药或复发的儿童LCH。以前没有关于其在成年LCH患者中作为一线单一疗法使用的报道。
