Myeloma of the central nervous system: association with high-risk chromosomal abnormalities, plasmablastic morphology and extramedullary manifestations.
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.
Fassas
Athanasios B-T
AB
Myeloma and Transplantation Research Center, Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USA. FassasAthanasios@uams.edu
Muwalla
Firas
F
Berryman
Tanya
T
Benramdane
Riad
R
Joseph
Lija
L
Anaissie
Elias
E
Sethi
Rajesh
R
Desikan
Raman
R
Siegel
David
D
Badros
Ashraf
A
Toor
Amir
A
Zangari
Maurizio
M
Morris
Christopher
C
Angtuaco
Edgardo
E
Mathew
Sajini
S
Wilson
Carla
C
Hough
Aubrey
A
Harik
Sami
S
Barlogie
Bart
B
Tricot
Guido
G
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
D013792:Thalidomide; D014750:Vincristine; D003907:Dexamethasone; D004317:Doxorubicin