Treatment of Epstein-Barr-virus-associated primary CNS B cell lymphoma with allogeneic T-cell immunotherapy and stem-cell transplantation

2005 
344 http://oncology.thelancet.com Vol 6 May 2005 An 8-year-old girl presented in October, 2003, with a 3-week history of headaches, vomiting, diplopia secondary to palsies in the IV and VI cranial nerves, dysarthria, and unsteady gait. Over the following 2 weeks, bulbar palsy progressed, and she developed quadraparesis and obtunded consciousness that needed intensive care with assisted ventilation. The patient did not have lymphadenopathy or hepatosplenomegaly. Gadolinium-enhanced cranial MRI showed moderate hydrocephalus and several cerebral and cerebellar ring-like lesions with no central liquefaction (figure 1A,B). Urgent right frontal external ventricular drainage was done. Cerebrospinal fluid taken during the operation showed no white cells, presence of normal proteins, and normal glucose concentrations; the fluid was negative on PCR, microscopy, and culture for bacteria and fungi. 40 380 copies/mL of Epstein-Barr virus (EBV) DNA were detected by PCR of cerebrospinal fluid, and 6 316 copies/mL were found in peripheral blood. Histological analysis of a biopsy sample of the right parietal brain obtained by image guidance showed an infiltrate of CD79a-positive CD30-positive ALK-1-negative, activated B lymphocytes with pleomorphic nuclei and prominent nucleoli. Cells expressed EBV latent membrane protein 1 (LMP1), EBV encoded small RNA (EBER, shown by in-situ hybridisation) and EBV DNA (shown by PCR). Chest and abdominal CT, and bonemarrow biopsy sample were unremarkable, confirming primary CNS EBV-associated, B-cell lymphoma. The patient had had recurrent bacterial respiratory-tract infections during childhood. Furthermore, at age 5 years she was admitted to hospital for 3 weeks because of severe chickenpox, and was absent from school for Lancet Oncol 2005; 6: 344-46
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