title: [Primary central nervous system post-transplant lymphoproliferative disorders].
abstract: The post-transplant lymphoproliferative disorders (PTLD) are a heterogeneous disease entity of lymphoid and plasmacytic proliferations that can occur after solid organ and bone marrow/stem cell transplantation. PTLD sometimes involves the central nervous system (CNS), but primary occurrence in central nervous system (PCNS-PTLD) is rare. The Epstein-Barr virus (EBV) plays a causative role, and up to 90% of the tumors are associated with this virus. Diagnosing PCNS-PTLD is often challenging based solely on computed tomography, magnetic resonance imaging, and physical findings; therefore, direct biopsy of the lesion is usually necessary to make a definitive diagnosis. The optimal therapy for PCNS-PTLD remains unknown. Dose reduction or discontinuation of immunosuppressive agents is effective for approximately half of PTLD patients, but not for most patients with PCNS-PTLD. It has been noted that CNS involvement is a poor prognostic factor, but early diagnosis and initiation of chemotherapy or radiotherapy seem critical for maximizing the likelihood of a favorable outcome.
authors.affiliation: Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine.
authors.forename: Masaya
authors.initials: M
authors.lastname: Honda
authors.forename: Michiaki
authors.initials: M
authors.lastname: Koga
authors.forename: Takashi
authors.initials: T
authors.lastname: Kanda
mesh_terms: D002490:Central Nervous System / Q000473:pathology*; D042241:Early Diagnosis; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D011183:Postoperative Complications / Q000175:diagnosis / Q000188:drug therapy*
chemical_list: D007166:Immunosuppressive Agents