title: Renal disease related to Waldenström macroglobulinaemia: incidence, pathology and clinical outcomes.
abstract: The incidence and prognostic impact of nephropathy related to Waldenström macroglobulinaemia (WM) is currently unknown. We performed a retrospective study to assess biopsy-confirmed WM-related nephropathy in a cohort of 1391 WM patients seen at a single academic institution. A total of 44 cases were identified, the estimated cumulative incidence was 5·1% at 15 years. There was a wide variation in kidney pathology, some directly related to the WM: amyloidosis (n = 11, 25%), monoclonal-IgM deposition disease/cryoglobulinaemia (n = 10, 23%), lymphoplasmacytic lymphoma infiltration (n = 8, 18%), light-chain deposition disease (n = 4, 9%) and light-chain cast nephropathy (n = 4, 9%), and some probably related to the WM: thrombotic microangiopathy (TMA) (n = 3, 7%), minimal change disease (n = 2, 5%), membranous nephropathy (n = 1, 2%) and crystal-storing tubulopathy (n = 1, 2%). The median overall survival in patients with biopsy-confirmed WM-related nephropathy was 11·5 years, shorter than for the rest of the cohort (16 years, P = 0·03). Survival was better in patients with stable or improved renal function after treatment (P = 0·05). Based on these findings, monitoring for renal disease in WM patients should be considered and a kidney biopsy pursued in those presenting with otherwise unexplained renal failure and/or nephrotic syndrome.
authors.affiliation: Bing Center for Waldenström Macroglobulinemia, Dana-Farber Cancer Institute, Boston, MA, USA.|Department of Internal Medicine/Haematology, St. Antonius Hospital, Nieuwegein, the Netherlands.
authors.forename: Josephine M
authors.initials: JM
authors.lastname: Vos
authors.affiliation: Bing Center for Waldenström Macroglobulinemia, Dana-Farber Cancer Institute, Boston, MA, USA.
authors.forename: Joshua
authors.initials: J
authors.lastname: Gustine
authors.affiliation: Department of Pathology, Brigham and Women's Hospital, Boston, MA, USA.|Harvard Medical School, Boston, MA, USA.
authors.forename: Helmut G
authors.initials: HG
authors.lastname: Rennke
authors.affiliation: Bing Center for Waldenström Macroglobulinemia, Dana-Farber Cancer Institute, Boston, MA, USA.
authors.forename: Zachary
authors.initials: Z
authors.lastname: Hunter
authors.affiliation: Bing Center for Waldenström Macroglobulinemia, Dana-Farber Cancer Institute, Boston, MA, USA.
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Manning
authors.affiliation: Bing Center for Waldenström Macroglobulinemia, Dana-Farber Cancer Institute, Boston, MA, USA.
authors.forename: Toni E
authors.initials: TE
authors.lastname: Dubeau
authors.affiliation: Bing Center for Waldenström Macroglobulinemia, Dana-Farber Cancer Institute, Boston, MA, USA.
authors.forename: Kirsten
authors.initials: K
authors.lastname: Meid
authors.affiliation: Department of Haematology, Academic Medical Centre, Amsterdam, the Netherlands.
authors.forename: Monique C
authors.initials: MC
authors.lastname: Minnema
authors.affiliation: Department of Haematology, UMC Utrecht Cancer Centre, Utrecht, the Netherlands.
authors.forename: Marie-Jose
authors.initials: MJ
authors.lastname: Kersten
authors.affiliation: Bing Center for Waldenström Macroglobulinemia, Dana-Farber Cancer Institute, Boston, MA, USA.|Harvard Medical School, Boston, MA, USA.
authors.forename: Steven P
authors.initials: SP
authors.lastname: Treon
authors.affiliation: Bing Center for Waldenström Macroglobulinemia, Dana-Farber Cancer Institute, Boston, MA, USA.|Harvard Medical School, Boston, MA, USA.
authors.forename: Jorge J
authors.identifier: http://orcid.org/0000-0001-9490-7532
authors.initials: JJ
authors.lastname: Castillo
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers; D001706:Biopsy; D005260:Female; D006801:Humans; D015994:Incidence; D007674:Kidney Diseases / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology* / Q000628:therapy; D007677:Kidney Function Tests; D008297:Male; D008875:Middle Aged; D063868:Patient Outcome Assessment; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D008258:Waldenstrom Macroglobulinemia / Q000150:complications* / Q000453:epidemiology* / Q000628:therapy
chemical_list: D015415:Biomarkers
keywords: Waldenström macroglobulinaemia; amyloidosis; epidemiology; haemodialysis; renal medicine