LCH 主要累及儿童。据报道,英国估计 LCH 年发病率为每百万 0-14 岁儿童中有 4.12 例。[8]Salotti J, Nanduri V, Pearce MS, et al. Incidence and clinical features of Langerhans cell histiocytosis in the UK and Ireland. Arch Dis Child. 2009 May;94(5):376-80.http://www.ncbi.nlm.nih.gov/pubmed/19060008?tool=bestpractice.com 目前尚无美国国家流行病学数据。
丹麦LCH患病率约为每 50,000 名儿童 1 例,年发病率为每百万 15 岁以下儿童 2 - 5 例。[9]Carstensen H, Ornvold K. The epidemiology of Langerhans cell histiocytosis in children in Denmark, 1975-89. Med Pediatr Oncol. 1993;21:387-88. 不过,瑞典的一项研究显示LCH年发病率更高,达到每百万儿童 8.9 例,且观察到多系统和单系统疾病发病率均增高。[10]Karis J, Bernstrand C, Fadeel B, et al. The incidence of Langerhans cell histiocytosis in children in Stockholm County, Sweden 1992-2001. Proceedings of the XIX meeting of the Histiocyte Society. Philadelphia, PA: Histiocyte Society; 2003:21. 由于 30% - 50% 的病例见于 15 岁以上的患者,因此,所有年龄组实际的年发病率接近每百万人 11 - 12 例。[11]Berry DH, Becton DL. Natural history of histiocytosis X. Hematol Oncol Clin North Am. 1987 Mar;1(1):23-34.http://www.ncbi.nlm.nih.gov/pubmed/3312147?tool=bestpractice.com 上述数字最可能低估实际情况,因为,许多有局部皮肤或骨骼疾病的患者或者未被确诊,或者未就诊。
15 岁以下患者确诊时的平均年龄是 30 个月,男性略高。[9]Carstensen H, Ornvold K. The epidemiology of Langerhans cell histiocytosis in children in Denmark, 1975-89. Med Pediatr Oncol. 1993;21:387-88. 其中大约 70% 的患者为单系统疾病,最常见的累及部位是骨骼。[12]Stuurman KE, Lau L, Doda W, et al. Evaluation of the natural history and long term complications of patients with Langerhans cell histiocystosis of bone. Proceedings of the XIX meeting of the Histiocyte Society. Philadelphia, PA: Histiocyte Society; 2003. 成人确诊时的平均年龄是 35 岁,10% >55 岁。 其中多数患者有多系统疾病,最常见的累及部位是皮肤和肺脏。[13]Arico M, Girschikofsky M, Genereau T, et al. Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society. Eur J Cancer. 2003 Nov;39(16):2341-8.http://www.ncbi.nlm.nih.gov/pubmed/14556926?tool=bestpractice.com