title: Lymphomatoid granulomatosis of central nervous system and lung driven by epstein barr virus proliferation: successful treatment with rituximab-containing chemotherapy.
abstract: Lymphomatoid granulomatosis (LYG) is a very rare Epstein-Barr virus (EBV) associated B-cell lymphoproliferative disorder. We report the case of a 41-year-old man who presented with fever and respiratory symptoms. Computed tomography showed multiple nodules in both lung fields. Polymerase chain reaction (PCR) analysis for EBV was positive in bronchoalveolar lavage and biopsy of lung node yielded a diagnosis of LYG, grade III. Shortly after initiation of treatment with agressive chemotherapy, neurological deterioration appeared. Neuroimaging findings revealed hydrocephalus and PCR analysis of the cerebrospinal fluid (CSF) was positive for EBV. Treatment with intravenous rituximab led to rapid reduction of EBV load in CSF, along with clinical and radiological improvement. After completion of treatment with immunochemotherapy, an autologous stem cell transplantation was performed. Patient stays in remission 18 months after diagnosis.
authors.affiliation: Department of Hematology, Hospital de Cabueñes, Gijon, Spain.
authors.forename: Ruben
authors.initials: R
authors.lastname: Fernandez-Alvarez
authors.affiliation: Department of Hematology, Hospital de Cabueñes, Gijon, Spain.
authors.forename: Me
authors.initials: M
authors.lastname: Gonzalez
authors.affiliation: Department of Hematology, Hospital de Cabueñes, Gijon, Spain.
authors.forename: Almudena
authors.initials: A
authors.lastname: Fernandez
authors.affiliation: Stem Cell Transplant Unit, Hospital Universitario Central de Asturias, Oviedo, Spain.
authors.forename: Ap
authors.initials: A
authors.lastname: Gonzalez-Rodriguez
authors.affiliation: Clinical Hematology Department, ICO-Hospital Germans Trias i Pujol, Barcelona, Spain.
authors.forename: Jm
authors.initials: J
authors.lastname: Sancho
authors.affiliation: Department of Pathology, Hospital de Cabueñes, Gijon, Spain.
authors.forename: Francisco
authors.initials: F
authors.lastname: Dominguez
authors.affiliation: Department of Hematology, Hospital de Cabueñes, Gijon, Spain.
authors.forename: Carmen
authors.initials: C
authors.lastname: Fernandez