title: Extramedullary Waldenström macroglobulinemia.
abstract: Disease assessment in Waldenstrom Macroglobulinemia (WM) is dependent on the percent involvement of B-cell neoplasm in the bone marrow and IgM paraprotein in the serum. A subset of patients also demonstrates extramedullary involvement, which is infrequently examined. The role of extramedullary involvement in the diagnosis and prognosis of WM is poorly understood. The purpose of this study is to report the characteristics of WM patients with extramedullary disease (EMD). Nine hundred and eight-five patients with WM were evaluated at one academic center and the presence of EMD was assessed in these patients. Forty-three (4.4%) patients were identified to have EMD. Nine (21%) patients presented with involvement at WM diagnosis, while 34 (79%) developed EMD post-therapy for WM. Most frequent EMD sites involved were pulmonary (30%), soft tissue (21%), cerebrospinal fluid (23%), renal (8%), and bone (9%). The median overall survival at 10 years was 79% (95% CI: 57-90%). This is the first study to describe the clinical characteristics, response and overall survival in patients with extramedullary WM. Further studies to define the molecular characteristics of this entity and mechanisms of its development are warranted.
authors.affiliation: Medical Oncology, Dana-Farber Cancer Institute, and Harvard Medical School, Boston, Massachusetts.
authors.forename: Ranjit
authors.initials: R
authors.lastname: Banwait
authors.forename: Yosra
authors.initials: Y
authors.lastname: Aljawai
authors.forename: Joseph
authors.initials: J
authors.lastname: Cappuccio
authors.forename: Serena
authors.initials: S
authors.lastname: McDiarmid
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Morgan
authors.forename: Houry
authors.initials: H
authors.lastname: Leblebjian
authors.forename: Aldo M
authors.initials: AM
authors.lastname: Roccaro
authors.forename: Jacob
authors.initials: J
authors.lastname: Laubach
authors.forename: Jorge J
authors.initials: JJ
authors.lastname: Castillo
authors.forename: Claudia
authors.initials: C
authors.lastname: Paba-Prada
authors.forename: Steven
authors.initials: S
authors.lastname: Treon
authors.forename: Robert
authors.initials: R
authors.lastname: Redd
authors.forename: Edie
authors.initials: E
authors.lastname: Weller
authors.forename: Irene M
authors.initials: IM
authors.lastname: Ghobrial
mesh_terms: D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001402:B-Lymphocytes / Q000276:immunology / Q000473:pathology*; D001853:Bone Marrow / Q000276:immunology / Q000473:pathology*; D001842:Bone and Bones / Q000276:immunology / Q000473:pathology*; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood; D007668:Kidney / Q000276:immunology / Q000473:pathology*; D008168:Lung / Q000276:immunology / Q000473:pathology*; D008198:Lymph Nodes / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016019:Survival Analysis; D008258:Waldenstrom Macroglobulinemia / Q000188:drug therapy / Q000276:immunology / Q000401:mortality / Q000473:pathology*
chemical_list: D000970:Antineoplastic Agents; D007075:Immunoglobulin M