郎格罕细胞组织细胞增生症46例临床研究  

The analysis of 46 patients with Langerhans cell histiocytosis

作　　者：徐酉华[1] 苏庸春[1] 刘筱梅[1] 

机构地区：[1]重庆医科大学儿童医院血液科,400014

出　　处：《重庆医学》2002年第11期1059-1060,共2页Chongqing medicine

摘　　要：目的　探讨郎格罕细胞组织细胞增生症的临床特点 ,提高临床诊治水平。方法　对近 8年来我院 4 6例郎格罕细胞组织细胞增生症进行临床回顾性研究分析。结果　 4 6例病人中最小发病年龄为 1个月 ,勒雪氏综合征的发病年龄都在 3岁以下 ;骨嗜酸性肉芽肿最小发病年龄 16个月。男多于女 ,但年龄在 12个月以下发病男女无差异。临床以发热、皮疹、骨损害、突眼、尿崩、耳溢等表现为主。病情轻重和预后与发病年龄密切相关。 4 6例中发现 1例伴海洋性贫血 ,1例伴G 6PD缺陷症。结论　郎格罕细胞组织细胞增生症临床表现多样化 ,病情轻重不一 ,典型皮疹印片有诊断意义。治疗采用手术刮除和化疗 ,合并尿崩时联合抗利尿激素使用 ,取得较好疗效。Objective To study the clinical features of the young patients with Langerhans cell histiocytosis (LCH) to improve the diagnosis level clinically. Methods Analyzed the clinical features of 46 patients with LCH diagnosed within the hospital in recent 8 years.Results Of 46 patients with LCH, the youngest was 1 month old. The patients with Letterer Siwe disease were all under 3 years old. Of the patiehts with eosinophilic granuloma , the youngest was 16 months old. Generally male patients are more than female patients except those under 12 month old who showed no sex difference. Clinically the fever, skin lesion, skeleton involvement, eye involvement and pituitary dysfunction are the most common manifestation. The severe degree and prognosis of the disease were associated with the onset age closely. Of the 46 patients with LCH, one case was associated with thalassemia and one with G 6PD. Conclusion The young patients with LCH have diverse manifestations which ranged from mild to severe degree. The biopsy of skin lesion is helpful for the diagnosis.

关 键 词：临床研究 郎格罕细胞组织细胞增生症 勒雪氏病 嗜酸性肉芽肿 

分 类 号：R551.1[医药卫生—血液循环系统疾病]