Myelomatous Involvement of the Central Nervous System.
Limited data exist with respect to the outcome and optimal treatment of patients with myelomatous involvement of the central nervous system (CNS).



Of 4060 patients with multiple myeloma (MM), evaluated at Mayo Clinic from 1998 to 2014, 29 (0.7%) had identifiable CNS involvement, established by the presence of atypical plasma cells in the cerebrospinal fluid (CSF) and/or identification of intraparenchymal or meningeal involvement on magnetic resonance imaging (MRI). A cohort of 87 MM patients without CNS disease served as the control group (1:3), matched by diagnosis date and gender.



Plasma cells were detected in the CSF in 87% and MRI findings consistent with CNS involvement were noted in 82% of the patients. A bone marrow plasma cell labeling index of ≥ 3%, the presence of disease at other extramedullary sites, or peripheral blood plasma cells of > 800 per 150,000 events were associated with an odds ratio of 7.1, 10.3, and 14, respectively, for the risk of CNS involvement. Overall survival (OS) from the diagnosis of MM was significantly shorter in the CNS-MM group (median 40 months; 95% confidence interval [CI], 24-56 months) than in the control group (median, 93 months; 95% CI, 67-129 months). OS was 3.4 months from the detection of CNS disease. Patients who underwent autologous stem cell transplantation after CNS involvement (n = 7) had a median OS of 19 months (95% CI, 10-67 months) from the detection of CNS involvement.



Myelomatous involvement of the CNS is a rare complication that portends a poor survival. Current therapeutic approaches appear to be largely ineffective for this subset of patients with MM.
Division of Hematology, Mayo Clinic, Rochester, MN.
Jonas
J
Paludo
Fourth Department of Internal Medicine-Hematology, Faculty of Medicine, University Hospital Hradec Kralove, Charles University in Prague, Hradec Kralove, Czech Republic.
Utkarsh
U
Painuly
Division of Hematology, Mayo Clinic, Rochester, MN.
Shaji
S
Kumar
Division of Hematology, Mayo Clinic, Rochester, MN.
Wilson I
WI
Gonsalves
Division of Hematology, Mayo Clinic, Rochester, MN.
Vincent
V
Rajkumar
Division of Hematology, Mayo Clinic, Rochester, MN.
Francis
F
Buadi
Division of Hematology, Mayo Clinic, Rochester, MN.
Martha Q
MQ
Lacy
Division of Hematology, Mayo Clinic, Rochester, MN.
Angela
A
Dispenzieri
Division of Hematology, Mayo Clinic, Rochester, MN.
Robert A
RA
Kyle
Division of Neurology, Mayo Clinic, Rochester, MN.
Michelle L
ML
Mauermann
Division of Hematology, Ottawa Hospital, University of Ottawa, Ottawa, ON, Canada.
Arleigh
A
McCurdy
Division of Hematology, Mayo Clinic, Rochester, MN.
David
D
Dingli
Division of Hematology, Mayo Clinic, Rochester, MN.
Ronald S
RS
Go
Division of Hematology, Mayo Clinic, Rochester, MN.
Suzanne R
SR
Hayman
Division of Hematology, Mayo Clinic, Rochester, MN.
Nelson
N
Leung
Division of Hematology, Mayo Clinic, Rochester, MN.
John A
JA
Lust
Division of Hematology, Mayo Clinic, Rochester, MN.
Yi
Y
Lin
Division of Hematology, Mayo Clinic, Rochester, MN.
Morie A
MA
Gertz
Division of Hematology, Mayo Clinic, Rochester, MN. Electronic address: kapoor.prashant@mayo.edu.
Prashant
P
Kapoor
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers / Q000134:cerebrospinal fluid; D001921:Brain / Q000473:pathology; D016543:Central Nervous System Neoplasms / Q000175:diagnosis / Q000453:epidemiology* / Q000556:secondary* / Q000628:therapy; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000175:diagnosis / Q000453:epidemiology* / Q000473:pathology* / Q000628:therapy; D011159:Population Surveillance; D016019:Survival Analysis; D016896:Treatment Outcome
D015415:Biomarkers
Extramedullary; High-risk; Leptomeningeal; Multiple myeloma; Outcome