title: Fatal lymphomatoid granulomatosis with primary CNS-involvement in an immunocompetent 80-year-old woman.
abstract: An 80-year-old woman presented with weight loss, fatigue, dizziness and a brain stem lesion. Extensive work-up revealed lymphomatoid granulomatosis (LYG) with primary clinical manifestation in the central nervous system (CNS), a rare Epstein-Barr virus-driven multisystem lymphoproliferative disorder, to be causative for the symptoms. Immunochemotherapy consisting of rituximab and temozolomide was started, but the disease progressed and the patient subsequently died. Histology, diagnostic criteria, differential diagnosis and treatment options for LYG with CNS involvement are discussed. This case demonstrates that LYG with CNS involvement may necessitate more aggressive treatment approaches than combination therapy with rituximab and temozolomide.
authors.affiliation: Department of Neurology, Friedrich-Alexander University of Erlangen-Nuremberg, Erlangen, Germany.
authors.forename: David G
authors.initials: DG
authors.lastname: Olmes
authors.affiliation: Institute of Pathology, Friedrich-Alexander University of Erlangen-Nuremberg, Erlangen, Germany.
authors.forename: Abbas
authors.initials: A
authors.lastname: Agaimy
authors.affiliation: Department of Neuroradiology, Friedrich-Alexander University of Erlangen-Nuremberg, Erlangen, Germany.
authors.forename: Stephan
authors.initials: S
authors.lastname: Kloska
authors.affiliation: Department of Neurology, Friedrich-Alexander University of Erlangen-Nuremberg, Erlangen, Germany.
authors.forename: Ralf A
authors.initials: RA
authors.lastname: Linker
mesh_terms: D000369:Aged, 80 and over; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002490:Central Nervous System / Q000473:pathology*; D016543:Central Nervous System Neoplasms / Q000188:drug therapy / Q000209:etiology / Q000628:therapy* / Q000821:virology; D003606:Dacarbazine / Q000031:analogs & derivatives / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D004854:Herpesvirus 4, Human; D006801:Humans; D008230:Lymphomatoid Granulomatosis / Q000188:drug therapy / Q000473:pathology / Q000628:therapy* / Q000821:virology; D000069283:Rituximab; D000077204:Temozolomide
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab; D003606:Dacarbazine; D000077204:Temozolomide