组织细胞增生症, 也称为朗格汉斯细胞组织细胞增多症(LCH), 正式名称为组织细胞增多症X, represents a group of rare disorders involving specific cells that normally have important roles as part of the immune system. 而LCH的病因尚不清楚, LCH经常表现得像癌症，因此由癌症专家治疗.

组织细胞增生症 is a generic name for a group of syndromes characterized by an abnormal increase in the number of certain immune cells called histiocytes. 这些细胞包括单核细胞、巨噬细胞和树突状细胞.

A histiocyte is a normal immune cell that is found in many parts of the body especially in the 骨 marrow, 血液流动, 皮肤, 肝脏, 肺部, 淋巴腺和脾脏. 在组织细胞增生症, the histiocytes move into tissues where they are not normally found and cause damage to those tissues. These proliferating immune cells may form tumors, which can affect various parts of the body.

LCH通常被归类为单一系统, 当疾病只影响身体的一个部位时, 或多系统, 当它影响到身体的多个部位时.  In children, histiocytosis usually involves the 骨s and may consist of single or multiple sites. 颅骨经常受到影响.  Children over five years of age usually have the single system disease, with just 骨 involvement. 幼儿，尤其是婴儿，更容易患多系统疾病.

大多数组织细胞增多症的病例影响1至15岁的儿童, 尽管所有年龄段的人都可能患上LCH. 发病率在5至10岁的儿童中达到高峰. 组织细胞增多症被认为每年大约会影响20万人中的1到2人. 组织细胞增多症的确切病因尚不清楚. 然而, recent studies indicate that it is caused by the development and expansion of an abnormal Langerhans cell that subsequently leads to the accumulation of other cells of the immune system, 导致身体不同部位的集合或肿瘤. 有些形式是遗传的.

组织细胞增生症症状

组织细胞增多症的第一个症状通常是头皮上出现皮疹，类似于摇篮帽. 可能会有骨痛、耳朵有分泌物、食欲不振和发烧. 有时胃又肿又痛. 偶尔, 大脑中一个被称为脑下垂体的区域受到影响, 这可能会导致孩子排出大量的尿液，并且非常口渴. 其他潜在的体征和症状包括:体重减轻, 黄疸, 呕吐, 一瘸一拐的, 身材矮小, 青春期延迟, 精神恶化, 头疼, 头晕, 癫痫发作, 突出的眼球, 和/或全身皮疹.

肿瘤在骨骼x光片上产生“穿孔”状. 有时，由于这些骨损伤，儿童会发生自发性骨折. 通常也会有系统性的参与, 哪一种会影响全身并引起皮疹, 肺部问题, 口香糖渗透, 淋巴腺肿胀, 荷尔蒙的问题, 脾和肝肿大, 和贫血.

组织细胞增多症诊断试验

诊断检查包括:活组织检查, in which a small sample of skin and/or 骨 is taken and examined under a microscope for abnormal cells; routine and sometimes specialized X-rays and scans of the 骨s, 头骨, and 肺部; and blood tests. These tests will help the doctor determine if the disease is the single system 或多系统 type.
An X-ray of the entire skeletal system may be done to determine how extensive the disease is and whether or not the systemic involvement is indicated.

组织细胞增生症治疗

取决于疾病的严重程度, LCH is often treated with chemotherapy and steroids to suppress the function of the immune system and the production of histiocytes. 治疗时间因儿童而异. 许多患者有资格参加国际和当地机构的试验.

放射治疗, 定向x射线治疗, 在某些情况下，有限的手术也可用于治疗骨损伤.

大多数出现组织细胞增多症的患者都能完全康复.  这种病有时会复发, so the patient will have regularly scheduled follow-up visits in the outpatient clinic as a precaution.

组织细胞增生症的研究

New ideas are being tested to determine the causes of LCH as well as why some patients respond better to treatment than others. 新的治疗方法正在开发中, 包括新型药物, as are approaches that direct antibodies or small molecules to the abnormal Langerhans cell while sparing the normal tissues.