Lipton J M
Pediatr Dermatol. 1983 Oct;1(2):112-20. doi: 10.1111/j.1525-1470.1983.tb01101.x.
The histiocytosis syndromes previously known, and often still referred to, as histiocytosis-X were originally categorized by many as malignant neoplasms. They have been treated as such with aggressive chemotherapy and radiation therapy. Although these modalities are still used, there are significant differences between the histiocytosis syndromes and the true malignant disease that suggest conservative management for many patients. The clinical course of malignant neoplasia is relentlessly progressive with virtually no survival in untreated patients. The histiocytosis syndromes are characterized by frequent spontaneous remissions and exacerbations, with varying morbidity and survival in untreated patients, depending on the extent of the disease. Pathologically, the lesions of histiocytosis appear as reactive infiltrates, possessing little of the cellular atypicality and homogeneity characteristic of malignancy. Although the etiology of these phenomena is unknown, histiocytosis syndromes appear to represents a reactive autoimmune disorder triggered by unknown stimuli.
组织细胞增多症综合征以前被知晓，并且现在常常仍被称为组织细胞增多症-X，最初许多人将其归类为恶性肿瘤。一直以来都用积极的化疗和放射疗法来治疗它们。尽管目前仍在使用这些治疗方式，但组织细胞增多症综合征与真正的恶性疾病之间存在显著差异，这表明对许多患者应采取保守治疗。恶性肿瘤的临床病程是持续进展的，未经治疗的患者几乎没有存活的可能。组织细胞增多症综合征的特点是频繁出现自发缓解和病情加重，未经治疗的患者的发病率和存活率各不相同，这取决于疾病的程度。在病理上，组织细胞增多症的病变表现为反应性浸润，几乎不具备恶性肿瘤所特有的细胞异型性和同质性。尽管这些现象的病因尚不清楚，但组织细胞增多症综合征似乎代表了一种由未知刺激引发的反应性自身免疫性疾病。
