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男性,16岁。以多饮多尿4年、右眼突出2年入院。体检:身长131cm,体重20kg。头顶骨多处可扪及椭圆形缺损和囊状肿块。右眼球突出。肋软骨呈串珠样隆起。心肺(-)。肝于右肋下1.5cm 扪及。外生殖器未发育。Hb8.5g,血胆固醇、钙,磷正常,尿糖(-),尿比重1.006～1.007。淋巴结活检示网状细胞显著增生。骨髓象示巨核、浆,网状细胞明显增多,并有脂肪细胞。X 线检查:颅骨示地图样病变,
Male, 16 years old. To drink more urine for 4 years, the right eye prominent 2 years hospitalization. Physical examination: length 131cm, weight 20kg. Parietal multiple palpable oval defects and cystic mass. Right eyeball prominent. Costal cartilage beaded like uplift. Cardiopulmonary (-). Liver in the right rib 1.5cm palpable. External genital undeveloped. Hb8.5g, blood cholesterol, calcium, phosphorus normal, urine sugar (-), urine specific gravity 1.006 ~ 1.007. Lymph node biopsy showed significant proliferation of reticulocytes. Bone marrow megakaryocyte, plasma, reticulocytes significantly increased, and fat cells. X-ray examination: skull showed pattern lesions,