Lymphoma and cerebral vasculitis in association with X-linked lymphoproliferative disease

作     者：Jia Zhu Yu Zhang Zi-Jun Zhen Yan Chen Juan Wang Rui-Qing Cai Xiao-Fei Sun 

作者机构：State Key Laboratory of Oncology in South China Department of Pediatric Oncology Sun Yat-sen University Cancer Center Department of Pathology Sun Yatsen University Cancer Center 

出 版 物：《Chinese Journal of Cancer》 (Chin. J. Cancer)

年 卷 期：2013年第32卷第12期

页      面：673-677页

核心收录：

学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学] 

主　　题：淋巴瘤 脑脊液 血管炎 关联性 淋巴组织 疾病 连锁 增生性 

摘      要：Lymphoma is seen in up to 30%of patients with X-linked lymphoproliferative disease(XLP),but cerebral vasculitis related with XLP after cure of Burkitt lymphoma is rarely *** describe a case of a 5-year-old boy with XLP who developed cerebral vasculitis two years after cure of Burkitt *** had Burkitt lymphoma at the age of 3 years and received chemotherapy(non-Hodgkin’s lymphomaBerlin-Frankfurt-Milan-90 protocol plus rituximab),which induced complete remission over the following two *** the age of 5 years,the patient first developed headache,vomiting,and then intellectual and motorial *** condition was not improved after anti-infection,dehydration,or dexamethasone *** tumor cells were found in his cerebrospinal *** resonance imaging showed multiple non-homogeneous,hypodense masses along the bilateral *** after biopsy revealed hyperplasia of neurogliocytes and vessels,accompanied by lymphocyte infiltration but no tumor cell *** aggressive treatment,his cognition and motor functions deteriorated in response to progressive cerebral *** patient is presently in a vegetative *** present this case to inform clinicians of association between lymphoma and immunodeficiency and explore an optimal treatment for lymphoma patients with compromised immune system.