Gramatovici R, D'Angio G J
Newfoundland Cancer Clinic, Health Sciences Centre, St. John's, Canada.
Med Pediatr Oncol. 1988;16(4):259-62. doi: 10.1002/mpo.2950160407.
Langerhans' cell histiocytosis (LCH) (previously histiocytosis X) is an infrequent disease with protean clinical manifestations and an unpredictable course. The role of radiotherapy in the soft tissue complications of LCH was evaluated in this retrospective study of 40 patients seen in two major Philadelphia institutions between 1970 and 1984. The patients were divided into two groups according to the extent of disease: unifocal versus multifocal. There was a higher rate of complete response in the unifocal form (4 of 12 patients), but this was associated with a high rate of spontaneous remissions (6 out of 12 patients). Multifocal LCH has a more aggressive course, and the major organs involved (lung, liver, and spleen) did not respond to radiotherapy. Eight patients had diabetes insipidus, and none of them responded to radiotherapy or chemotherapy. Autopsies were performed in two patients with diabetes insipidus who died of disease. There were no pathologic changes in the pituitary gland or stalk, hypothalamus, or supraoptic nuclei.
朗格汉斯细胞组织细胞增多症（LCH）（以前称为组织细胞增多症X）是一种罕见疾病，临床表现多样，病程不可预测。本回顾性研究对1970年至1984年间在费城两家主要机构就诊的40例患者进行了评估，以探讨放疗在LCH软组织并发症中的作用。根据疾病范围将患者分为两组：单病灶组与多病灶组。单病灶形式的完全缓解率较高（12例患者中有4例），但这与高自发缓解率相关（12例患者中有6例）。多病灶LCH病程更具侵袭性，受累的主要器官（肺、肝和脾）对放疗无反应。8例患者患有尿崩症，他们均对放疗或化疗无反应。对2例死于该病的尿崩症患者进行了尸检。垂体、垂体柄、下丘脑或视上核均无病理改变。
