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一般认为,有多系统损害、特别是重要器官受累的儿童组织细胞增生症X(histiocytosis X,HX)的预后不良。2岁以下儿童有肺、肝或骨髓损害者(Letterer-Siwe病)死亡率达50～70%。过去认为本病是一种恶性疾患,应用单个或多个药物联合治疗,包括肾上腺皮质激素和烷化剂。然而单个器官受累的儿童HX却常有自然痊愈者。由于组织学、免疫组织化学和免疫学不能鉴别HX单器官受累与多系统受累,所以,可能也有多系统受累者自然恢复的。作者观察了经皮肤活检证实为HX的2例婴儿。例1出生后24小时内即出现皮疹,X线检查有肺间质阴影增多,肺功能检查示肺容积减少。诊为HX时,病几
It is generally accepted that there is a poor prognosis for multiple systemic injuries, especially histiocytosis X (HX), a major organ involvement. Children under 2 years of age have lung, liver or bone marrow damage (Letterer-Siwe disease) mortality rate of 50 to 70%. In the past that the disease is a malignant disease, the application of single or multiple drug combination therapy, including adrenal cortex hormones and alkylating agents. However, children with single organ involvement are often naturally healed HX. Because histology, immunohistochemistry and immunology do not identify HX single organ involvement and multiple system involvement, there may also be multiple system involvement that naturally recovers. The authors looked at 2 infants confirmed by skin biopsy as HX. Example 1 within 24 hours after birth that there rash, X-ray examination increased interstitial lung shadow, lung function tests showed a decrease in lung volume. When diagnosed as HX, the disease a few