勒-雪氏症又称婴幼儿网状内皮细胞增生症,本症多见于2岁以下的婴幼儿,男孩较多。患者全身非充脂性网状内皮细胞广泛性增生,正常组织遭受破坏,造成出血和坏死,尤以脾及皮肤为显著;病势非常凶险,病程短至半月,长至2年余。临床表现多有皮疹、发热、中耳炎、骨骼缺损、肝、脾、淋巴结肿大、肺部浸润、进行性贫血,部分患儿可有腹泻及黄疸等。本病是一种少见的疾病,近年来国内外文献有零星报道,现将我们见到的1例报告如下。 Le - Schizophrenia, also known as infantile reticuloendotheliosis, the disease more common in infants under 2 years of age, more boys. Patients with non-hyperplastic reticuloendothelial endothelial cell proliferation, destruction of normal tissue, resulting in bleeding and necrosis, especially in the spleen and skin were significant; the disease is very dangerous, the disease as short as half a month, up to 2 years. Clinical manifestations and more rashes, fever, otitis media, bone defects, liver, spleen, lymph nodes, pulmonary infiltrates, progressive anemia, some children may have diarrhea and jaundice. The disease is a rare disease, sporadic reports at home and abroad in recent years, now we see a report as follows.