中国实用儿科杂志2011，Vol.26Issue(2)：121-124,4.
自身免疫性淋巴细胞增殖综合征1例报告并文献复习
One case report and literature review of autoimmune lymphoproliferative syndrome
黄礼彬 1张丽丹 1柯志勇 1徐玲玲 1刘茵 2王卓 3罗学群1
作者信息
- 1. 中山大学附属第一医院儿科,广州,510080
- 2. 中山大学附属第一医院检验科,广州,510080
- 3. 中山大学附属第一医院病理科,广州,510080
- 折叠
摘要
Abstract
Objective To report the clinical features, its treatment and follow-up of one autoimmune lymphoproliferative syndrome (ALPS), and enhance the recognition of this rare disease in China. Methods The clinical features, treatment and follow-up data of a case diagnosed as ALPS in our hospital were analyzed, and related literature was reviewed. Resuits This 1-year-and- 11- month old boy presented with pancytopenia, hepatosplenomegaly, and lymphadenopathy, as well as the clinical features of glomerulonephritis and inflammatory bowel disease.The percentage of CD3+CD4CD8 double negative T (DNT) cell increased markedly and had positive autoantibodies, such as PAIgG, ANA, ANCA and insulin autoantibody.The genes of Fas, Fas ligand and Casp10 were sequenced but no mutation was found. Glucocorticosteroid in combination with other immunosuppressive agents was effective in short term, but relapses occurred when glucocorticosteroid was tapered. Conclusion The diagnosis of autoimmune lymphoproliferative syndrome in this case is clinically proper. It would reduce misdiagnosis rate if the recognition of this disease is enhanced.关键词
自身免疫性/淋巴细胞增殖综合征/肾小球肾炎/炎症性肠病分类
医药卫生引用本文复制引用
黄礼彬,张丽丹,柯志勇,徐玲玲,刘茵,王卓,罗学群..自身免疫性淋巴细胞增殖综合征1例报告并文献复习[J].中国实用儿科杂志,2011,26(2):121-124,4.
