title: Myeloma of the central nervous system: association with high-risk chromosomal abnormalities, plasmablastic morphology and extramedullary manifestations.
abstract: Involvement of the central nervous system (CNS) by multiple myeloma, as defined by the detection of malignant plasma cells in the cerebrospinal fluid in the presence of suggestive symptoms, is considered extremely rare. We report on the characteristics of 18 such patients diagnosed and treated at the University of Arkansas over the last 10 years for an overall incidence of approximately 1%. Their evaluation revealed association of CNS involvement with unfavourable cytogenetic abnormalities (especially translocations and deletion of the chromosome 13), high tumour mass, plasmablastic morphology, additional extramedullary myeloma manifestations and circulating plasma cells. The presence of these features should alert clinicians to the possibility of CNS involvement. The outcome of these patients was extremely poor despite the use of aggressive local and systemic treatment including autologous stem cell transplants. Given this universally poor prognosis, the application of allogeneic transplants should be studied in this clinical setting.
authors.affiliation: Myeloma and Transplantation Research Center, Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USA. FassasAthanasios@uams.edu
authors.forename: Athanasios B-T
authors.initials: AB
authors.lastname: Fassas
authors.forename: Firas
authors.initials: F
authors.lastname: Muwalla
authors.forename: Tanya
authors.initials: T
authors.lastname: Berryman
authors.forename: Riad
authors.initials: R
authors.lastname: Benramdane
authors.forename: Lija
authors.initials: L
authors.lastname: Joseph
authors.forename: Elias
authors.initials: E
authors.lastname: Anaissie
authors.forename: Rajesh
authors.initials: R
authors.lastname: Sethi
authors.forename: Raman
authors.initials: R
authors.lastname: Desikan
authors.forename: David
authors.initials: D
authors.lastname: Siegel
authors.forename: Ashraf
authors.initials: A
authors.lastname: Badros
authors.forename: Amir
authors.initials: A
authors.lastname: Toor
authors.forename: Maurizio
authors.initials: M
authors.lastname: Zangari
authors.forename: Christopher
authors.initials: C
authors.lastname: Morris
authors.forename: Edgardo
authors.initials: E
authors.lastname: Angtuaco
authors.forename: Sajini
authors.initials: S
authors.lastname: Mathew
authors.forename: Carla
authors.initials: C
authors.lastname: Wilson
authors.forename: Aubrey
authors.initials: A
authors.lastname: Hough
authors.forename: Sami
authors.initials: S
authors.lastname: Harik
authors.forename: Bart
authors.initials: B
authors.lastname: Barlogie
authors.forename: Guido
authors.initials: G
authors.lastname: Tricot
mesh_terms: D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001921:Brain / Q000473:pathology; D016543:Central Nervous System Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D025063:Chromosome Disorders; D003131:Combined Modality Therapy; D003907:Dexamethasone / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D010950:Plasma Cells / Q000473:pathology; D011379:Prognosis; D013792:Thalidomide / Q000008:administration & dosage; D014182:Transplantation, Autologous; D014184:Transplantation, Homologous; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D013792:Thalidomide; D014750:Vincristine; D003907:Dexamethasone; D004317:Doxorubicin