title: Richter transformation in the brain from chronic lymphocytic leukemia.
abstract: Richter syndrome (RS) involves the development of an aggressive lymphoma in patients with chronic lymphocytic leukemia (CLL). Diffuse large B-cell lymphoma (DLBCL) is the most common type of RS. Extranodal RS occasionally occurs; however, isolated lesions in the central nervous system (CNS) of RS are rarely seen and the features have not been well described. We describe a Japanese patient who developed isolated involvements of the parenchyma of the CNS as a manifestation of RS two years after the initial diagnosis of CLL. DLBCL in the cerebrum was confirmed to be clonally related to the CLL cells by immunoglobulin heavy chain (IGH) gene analysis, utilizing the identical VH-D-J regions with additional mutations in the IGH variable region.
authors.affiliation: Department of Biomedical Laboratory Sciences, Shinshu University School of Medicine, Matsumoto, Japan. fumishi@shinshu-u.ac.jp
authors.forename: Fumihiro
authors.initials: F
authors.lastname: Ishida
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Nakazawa
authors.forename: Yuka
authors.initials: Y
authors.lastname: Takezawa
authors.forename: Kazuyuki
authors.initials: K
authors.lastname: Matsuda
authors.forename: Naoko
authors.initials: N
authors.lastname: Asano
authors.forename: Kenji
authors.initials: K
authors.lastname: Sano
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Sakai
authors.forename: Toshiro
authors.initials: T
authors.lastname: Ito
mesh_terms: D000368:Aged; D001706:Biopsy; D001921:Brain / Q000473:pathology; D001932:Brain Neoplasms / Q000175:diagnosis / Q000556:secondary* / Q000628:therapy; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D012008:Recurrence; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D007143:Immunoglobulin Heavy Chains