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 reported.We describe a case of a 5-year-old boy with XLP who developed cerebral vasculitis two years after cure of Burkitt lymphoma.He 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 years.At the age of 5 years,the patient first developed headache,vomiting,and then intellectual and motorial retrogression.His condition was not improved after anti-infection,dehydration,or dexamethasone therapy.No tumor cells were found in his cerebrospinal fluid.Magnetic resonance imaging showed multiple non-homogeneous,hypodense masses along the bilateral cortex.Pathology after biopsy revealed hyperplasia of neurogliocytes and vessels,accompanied by lymphocyte infiltration but no tumor cell infiltration.Despite aggressive treatment,his cognition and motor functions deteriorated in response to progressive cerebral changes.The patient is presently in a vegetative state.We present this case to inform clinicians of association between lymphoma and immunodeficiency and explore an optimal treatment for lymphoma patients with compromised immune system.
Lymphoma is seen in up to 30% of patients with X-linked lymphoproliterative disease （XLP）, DUt cerebral vasculitis related with XLP after cure of Burkitt lymphoma is rarely reported. We describe a case of a 5-year-old boy with XLP who developed cerebral vasculitis two years after cure of Burkitt lymphoma. He had Burkitt lymphoma at the age of 3 years and received chemotherapy （non-Hodgkin＇s lymphoma- Berlin-Frankfurt-Milan-90 protocol plus rituximab）, which induced complete remission over the following two years. At the age of 5 years, the patient first developed headache, vomiting, and then intellectual and motorial retrogression. His condition was not improved after anti-infection, dehydration, or dexamethasone therapy. No tumor cells were found in his cerebrospinal fluid. Magnetic resonance imaging showed multiple non-homogeneous, hypodense masses along the bilateral cortex. Pathology after biopsy revealed hyperplasia of neurogliocytes and vessels, accompanied by lymphocyte infiltration but no tumor cell infiltration. Despite aggressive treatment, his cognition and motor functions deteriorated in response to progressive cerebral changes. The patient is presently in a vegetative state. We present this case to inform clinicians of association between lymphoma and immunodeficiency and explore an optimal treatment for lymphoma patients with compromised immune system.
关键词
淋巴瘤
脑脊液
血管炎
关联性
淋巴组织
疾病
连锁
增生性
Burkitt lymphoma, cerebral vasculitis, X-linked lymphoproliferative disease, Epstein-Barr virus
