title: Incidence and clinical features of extramedullary multiple myeloma in patients who underwent stem cell transplantation.
abstract: Extramedullary disease (EMD), defined as an infiltrate of clonal plasma cells at an anatomic site distant from the bone marrow, is an uncommon manifestation of multiple myeloma. Six hundred and sixty-three consecutive patients with multiple myeloma who underwent stem cell transplantation between January 2005 and December 2011 were assessed for the presence of EMD. A cohort of 55 patients with biopsy-proven EMD was identified, comprising 8·3% of the total study population. EMD was present at the time of diagnosis in 14·5% of cases and at the time of relapse in 76% of patients. The most common EMD presentations at relapse were liver involvement and pleural effusions. EMD specimens had high expression of CD44 (92%) and moderate expression of CXCR4. The median overall survival from time of myeloma diagnosis was 4·1 years (95% CI: 3·1, 5·1) and the median overall survival from time of EMD diagnosis was 1·3 years (95% CI: 0·8, 2·3). This report demonstrates that the incidence of EMD has not increased with the introduction of novel agents and stem cell transplantation. The most common EMD presentations in the relapsed setting were liver and pleural fluid. The presence of CD44 and CXCR4 expression may represent new markers of EMD that warrant further investigation.
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.|Beth Israel Deaconess, Harvard Medical School, Boston, MA, USA.
authors.forename: Mathew
authors.initials: M
authors.lastname: Weinstock
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Yosra
authors.initials: Y
authors.lastname: Aljawai
authors.affiliation: Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Morgan
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Jacob
authors.initials: J
authors.lastname: Laubach
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Muriel
authors.initials: M
authors.lastname: Gannon
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Aldo M
authors.initials: AM
authors.lastname: Roccaro
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Cindy
authors.initials: C
authors.lastname: Varga
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Constantine S
authors.initials: CS
authors.lastname: Mitsiades
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Claudia
authors.initials: C
authors.lastname: Paba-Prada
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Robert
authors.initials: R
authors.lastname: Schlossman
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Nikhil
authors.initials: N
authors.lastname: Munshi
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Kenneth C
authors.initials: KC
authors.lastname: Anderson
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Paul P
authors.initials: PP
authors.lastname: Richardson
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Edie
authors.initials: E
authors.lastname: Weller
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Irene M
authors.initials: IM
authors.lastname: Ghobrial
mesh_terms: D001706:Biopsy; D002490:Central Nervous System / Q000473:pathology; D005260:Female; D006258:Head and Neck Neoplasms / Q000209:etiology / Q000473:pathology; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007150:Immunohistochemistry; D015994:Incidence; D008198:Lymph Nodes / Q000473:pathology; D008297:Male; D009101:Multiple Myeloma / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology / Q000401:mortality / Q000628:therapy; D049268:Positron-Emission Tomography; D016896:Treatment Outcome
keywords: CD44; extramedullary; multiple myeloma; novel agents; stem cell transplantation
references.citation: N Engl J Med. 1996 Jul 11;335(2):91-7
references.pmid: 8649495
references.citation: J Clin Oncol. 1994 Nov;12(11):2398-404
references.pmid: 7964956
references.citation: Haematologica. 2012 Nov;97(11):1761-7
references.pmid: 22689675
references.citation: Br J Haematol. 1996 May;93(2):345-51
references.pmid: 8639427
references.citation: Blood. 2009 Sep 3;114(10):2068-76
references.pmid: 19443657
references.citation: J Clin Oncol. 2006 Jan 20;24(3):431-6
references.pmid: 16365178
references.citation: Leukemia. 2011 Jun;25(6):906-8
references.pmid: 21350560
references.citation: Ann Intern Med. 1989 Apr 1;110(7):521-5
references.pmid: 2647015
references.citation: Ann Hematol. 2012 Jul;91(7):1031-7
references.pmid: 22286070
references.citation: Ann Oncol. 2007 Jan;18(1):7-12
references.pmid: 16790518
references.citation: Leukemia. 2013 Oct;27(10):2075-7
references.pmid: 23728080
references.citation: N Engl J Med. 2007 Nov 22;357(21):2123-32
references.pmid: 18032762
references.citation: J Clin Oncol. 2011 Oct 1;29(28):3805-12
references.pmid: 21900099
references.citation: Blood. 1996 Oct 15;88(8):3101-8
references.pmid: 8874209
references.citation: N Engl J Med. 2005 Jun 16;352(24):2487-98
references.pmid: 15958804
references.citation: Ann Oncol. 2010 Feb;21(2):325-330
references.pmid: 19633044
references.citation: Leuk Lymphoma. 2013 Jun;54(6):1135-41
references.pmid: 23210572
references.citation: Cytometry B Clin Cytom. 2013 Jul-Aug;84(4):222-8
references.pmid: 23450831
references.citation: Br J Haematol. 2002 Feb;116(2):273-7
references.pmid: 11841427