title: IgM myeloma: A multicenter retrospective study of 134 patients.
abstract: IgM myeloma is a rare hematologic malignancy for which the clinicopathological features and patient outcomes have not been extensively studied. We carried out a multicenter retrospective study in patients with diagnosis of IgM myeloma defined by >10% marrow involvement by monoclonal plasma cells, presence of an IgM monoclonal paraproteinemia of any size, and anemia, renal dysfunction, hypercalcemia, lytic lesions and/or t(11;14) identified by FISH. A total of 134 patients from 20 centers were included in this analysis. The median age at diagnosis was 65.5 years with a male predominance (68%). Anemia, renal dysfunction, elevated calcium and skeletal lytic lesions were found in 37, 43, 19, and 70%, respectively. The median serum IgM level was 2,895 mg dL-1with 19% of patients presenting with levels >6,000 mg dL-1. International Staging System (ISS) stages 1, 2, and 3 were seen in 40 (33%), 54 (44%), and 29 (24%) of patients, respectively. The malignant cells expressed CD20 (58%) and cyclin D1 (67%), and t(11;14) was the most common cytogenetic finding (39%). The median overall survival (OS) was 61 months. Higher ISS score was associated with worse survival (P = 0.02). Patients with IgM myeloma present with similar characteristics and outcomes as patients with more common myeloma subtypes.
authors.affiliation: Dana-Farber Cancer Institute and Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Jorge J
authors.identifier: http://orcid.org/0000-0001-9490-7532
authors.initials: JJ
authors.lastname: Castillo
authors.affiliation: Jagiellonian University Medical College, Krakow, Poland.
authors.forename: Artur
authors.initials: A
authors.lastname: Jurczyszyn
authors.affiliation: Masaryk University, Brno, Czech Republic.
authors.forename: Lucie
authors.initials: L
authors.lastname: Brozova
authors.affiliation: Professor Edgar Santos University Hospital, Salvador, Brazil.
authors.forename: Edvan
authors.initials: E
authors.lastname: Crusoe
authors.affiliation: Jagiellonian University Medical College, Krakow, Poland.
authors.forename: Jacek
authors.initials: J
authors.lastname: Czepiel
authors.affiliation: University Hospital of Salamanca, Salamanca, Spain.
authors.forename: Julio
authors.initials: J
authors.lastname: Davila
authors.affiliation: Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Angela
authors.initials: A
authors.lastname: Dispenzieri
authors.affiliation: Nantes University Hospital, Nantes, France.
authors.forename: Marion
authors.initials: M
authors.lastname: Eveillard
authors.affiliation: Washington University School of Medicine, Saint Louis, Missouri, USA.
authors.forename: Mark A
authors.initials: MA
authors.lastname: Fiala
authors.affiliation: Dana-Farber Cancer Institute and Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Irene M
authors.initials: IM
authors.lastname: Ghobrial
authors.affiliation: Le Scotte Hospital, Siena, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Gozzetti
authors.affiliation: Dana-Farber Cancer Institute and Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Joshua N
authors.initials: JN
authors.lastname: Gustine
authors.affiliation: University of Ostrava and Faculty Hospital Ostrava, Ostrava, Czech Republic.
authors.forename: Roman
authors.initials: R
authors.lastname: Hajek
authors.affiliation: Santa Casa de Misericordia Hospital, Sao Paulo, Brazil.
authors.forename: Vania
authors.initials: V
authors.lastname: Hungria
authors.affiliation: Masaryk University, Brno, Czech Republic.
authors.forename: Jiri
authors.initials: J
authors.lastname: Jarkovsky
authors.affiliation: Weill Cornell Medical College, New York, New York, USA.
authors.forename: David
authors.initials: D
authors.lastname: Jayabalan
authors.affiliation: Dana-Farber Cancer Institute and Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Jacob P
authors.initials: JP
authors.lastname: Laubach
authors.affiliation: Jagiellonian University Medical College, Krakow, Poland.
authors.forename: Barbara
authors.initials: B
authors.lastname: Lewicka
authors.affiliation: Charles University Hospital, Hradec Kralove, Czech Republic.
authors.forename: Vladimir
authors.initials: V
authors.lastname: Maisnar
authors.affiliation: The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
authors.forename: Elisabet E
authors.initials: EE
authors.lastname: Manasanch
authors.affiliation: Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Philippe
authors.initials: P
authors.lastname: Moreau
authors.affiliation: Dana-Farber Cancer Institute and Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Morgan
authors.affiliation: Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Hareth
authors.initials: H
authors.lastname: Nahi
authors.affiliation: Weill Cornell Medical College, New York, New York, USA.
authors.forename: Ruben
authors.initials: R
authors.lastname: Niesvizky
authors.affiliation: Dana-Farber Cancer Institute and Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Claudia
authors.initials: C
authors.lastname: Paba-Prada
authors.affiliation: Palacky University Hospital, Olomouc, Czech Republic.
authors.forename: Tomas
authors.initials: T
authors.lastname: Pika
authors.affiliation: University Hospital Brno, Brno, Czech Republic.
authors.forename: Ludek
authors.initials: L
authors.lastname: Pour
authors.affiliation: Rhode Island Hospital, Alpert Medical School of Brown University, Providence, Rhode Island, USA.
authors.forename: John L
authors.initials: JL
authors.lastname: Reagan
authors.affiliation: Dana-Farber Cancer Institute and Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Paul G
authors.initials: PG
authors.lastname: Richardson
authors.affiliation: The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
authors.forename: Jatin
authors.initials: J
authors.lastname: Shah
authors.affiliation: Charles University Hospital, Prague, Czech Republic.
authors.forename: Ivan
authors.initials: I
authors.lastname: Spicka
authors.affiliation: Washington University School of Medicine, Saint Louis, Missouri, USA.
authors.forename: Ravi
authors.initials: R
authors.lastname: Vij
authors.affiliation: Warsaw Medical University, Warsaw, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Waszczuk-Gajda
authors.affiliation: Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Morie A
authors.initials: MA
authors.lastname: Gertz
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers; D001853:Bone Marrow / Q000378:metabolism / Q000473:pathology; D001842:Bone and Bones / Q000473:pathology; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D010950:Plasma Cells / Q000378:metabolism / Q000473:pathology; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D007075:Immunoglobulin M