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朗格汉斯细胞组织细胞增多症(LCH)是一类罕见的、症状复杂多变、以组织细胞浸润为特征的疾病,可累及多个器官系统。LCH是一种以骨髓树突前体细胞单克隆增生导致器官功能障碍为特征的肿瘤,界于良恶性肿瘤之间。LCH临床表现具有异质性强、复杂多变的特点,病变可为局灶性或弥漫性浸润,轻者仅累及皮肤、单骨或多骨损害,部分患者伴有尿崩症,重者可累及多器官系统,如肺脏、脾脏、肝脏以及造血系统。骨是LCH最常受累的部位,骨骼影像学检查可见单部位或多部
Langerhans cell histiocytosis (LCH) is a rare class of diseases characterized by complex and variable symptoms that are characterized by histiocytic infiltrates and can involve multiple organ systems. LCH is a tumor characterized by organ dysfunction resulting from monoclonal proliferation of myeloid dendritic precursor cells, which is found in benign and malignant tumors. LCH clinical manifestations of heterogeneity, complex and changing characteristics of lesions can be focal or diffuse infiltration, the only light involving the skin, bone or multiple bone damage, some patients with diabetes insipidus, severe cases may be Involves multiple organ systems, such as the lungs, spleen, liver and hematopoietic system. Bone is the most common site of involvement of the LCH, with single or multiple images of the bone seen on the bone