title: Kidney diseases associated with monoclonal immunoglobulin M-secreting B-cell lymphoproliferative disorders: a case series of 35 patients.
abstract: BACKGROUND
  Kidney diseases associated with immunoglobulin M (IgM) monoclonal gammopathy are poorly described, with few data for patient outcomes and renal response.
  
  
  METHODS
  Case series.
  
  
  METHODS
  35 patients from 8 French departments of nephrology were retrospectively studied. Inclusion criteria were: (1) detectable serum monoclonal IgM, (2) estimated glomerular filtration rate (eGFR) < 60mL/min/1.73m(2) and/or proteinuria with protein excretion > 0.5g/d and/or microscopic hematuria, and (3) kidney biopsy showing monoclonal immunoglobulin deposits and/or lymphomatous B-cell renal infiltration. All patients received chemotherapy, including rituximab-based regimens in 8 cases.
  
  
  METHODS
  Patients were classified into 3 groups according to renal pathology: glomerular AL amyloidosis (group 1; n=11), nonamyloid glomerulopathies (group 2; n=15, including 9 patients with membranoproliferative glomerulonephritis), and tubulointerstitial nephropathies (group 3; n=9, including cast nephropathy in 5, light-chain Fanconi syndrome in 3, and isolated tumor infiltration in 1).
  
  
  RESULTS
  Posttreatment hematologic response (≥50% reduction in serum monoclonal IgM and/or free light chain level) and renal response (≥50% reduction in 24-hour proteinuria or eGFR≥30mL/min/1.73m(2) in patients with glomerular and tubulointerstitial disorders, respectively).
  
  
  RESULTS
  Nephrotic syndrome was observed in 11 and 6 patients in groups 1 and 2, respectively. Patients in group 3 presented with acute kidney injury (n=7) and/or proximal tubular dysfunction (n=3). Waldenström macroglobulinemia was present in 26 patients (8, 12, and 6 in groups 1, 2, and 3, respectively). Significant lymphomatous interstitial infiltration was observed in 18 patients (4, 9, and 5 patients, respectively). Only 9 of 29 evaluable patients had systemic signs of symptomatic hematologic disease (2, 5, and 2, respectively). In groups 1, 2, and 3, respectively, hematologic response was achieved after first-line treatment in 3 of 9, 9 of 10, and 5 of 6 evaluable patients, while renal response occurred in 5 of 10, 9 of 15, and 5 of 8 evaluable patients.
  
  
  CONCLUSIONS
  Retrospective study; insufficient population to establish the impact of chemotherapy.
  
  
  CONCLUSIONS
  IgM monoclonal gammopathy is associated with a wide spectrum of renal manifestations, with an under-recognized frequency of tubulointerstitial disorders.
authors.affiliation: Centre national de référence maladies rares amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU Poitiers, Université de Poitiers, Poitiers, France; Department of Nephrology, Hôpital Jean Bernard, CHU Poitiers, Université de Poitiers, Poitiers, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Chauvet
authors.affiliation: Centre national de référence maladies rares amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU Poitiers, Université de Poitiers, Poitiers, France; Department of Nephrology, Hôpital Jean Bernard, CHU Poitiers, Université de Poitiers, Poitiers, France; CNRS UMR 6101, Université de Limoges, Limoges, France. Electronic address: f.bridoux@chu-poitiers.fr.
authors.forename: Frank
authors.initials: F
authors.lastname: Bridoux
authors.affiliation: Centre national de référence maladies rares amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU Poitiers, Université de Poitiers, Poitiers, France; Department of Nephrology, Hôpital Jean Bernard, CHU Poitiers, Université de Poitiers, Poitiers, France.
authors.forename: Laure
authors.initials: L
authors.lastname: Ecotière
authors.affiliation: Centre national de référence maladies rares amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU Poitiers, Université de Poitiers, Poitiers, France; Department of Nephrology, Hôpital Jean Bernard, CHU Poitiers, Université de Poitiers, Poitiers, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Javaugue
authors.affiliation: Centre national de référence maladies rares amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU Poitiers, Université de Poitiers, Poitiers, France; CNRS UMR 6101, Université de Limoges, Limoges, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Sirac
authors.affiliation: Department of Immunology and Hematology, Hôpital Saint-Louis AP-HP, Paris, France.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Arnulf
authors.affiliation: Centre national de référence maladies rares amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU Poitiers, Université de Poitiers, Poitiers, France; Department of Nephrology, Hôpital Jean Bernard, CHU Poitiers, Université de Poitiers, Poitiers, France.
authors.forename: Antoine
authors.initials: A
authors.lastname: Thierry
authors.affiliation: Centre national de référence maladies rares amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU Poitiers, Université de Poitiers, Poitiers, France; Department of Pathology, Hôpital Jean Bernard, CHU Poitiers, Université de Poitiers, Poitiers, France.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Quellard
authors.affiliation: Department of Pathology, Hôpital Jean Bernard, CHU Poitiers, Université de Poitiers, Poitiers, France.
authors.forename: Serge
authors.initials: S
authors.lastname: Milin
authors.affiliation: Centre national de référence maladies rares amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU Poitiers, Université de Poitiers, Poitiers, France; CNRS UMR 6101, Université de Limoges, Limoges, France.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Bender
authors.affiliation: Centre national de référence maladies rares amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU Poitiers, Université de Poitiers, Poitiers, France; Department of Pathology, Hôpital Jean Bernard, CHU Poitiers, Université de Poitiers, Poitiers, France.
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Goujon
authors.affiliation: Centre national de référence maladies rares amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU Poitiers, Université de Poitiers, Poitiers, France; CNRS UMR 6101, Université de Limoges, Limoges, France; Department of Hematology, CHU Limoges, Université de Limoges, Limoges, France.
authors.forename: Arnaud
authors.initials: A
authors.lastname: Jaccard
authors.affiliation: Department of Immunology and Hematology, Hôpital Saint-Louis AP-HP, Paris, France.
authors.forename: Jean-Paul
authors.initials: JP
authors.lastname: Fermand
authors.affiliation: Centre national de référence maladies rares amylose AL et autres maladies à dépôts d'immunoglobulines monoclonales, CHU Poitiers, Université de Poitiers, Poitiers, France; Department of Nephrology, Hôpital Jean Bernard, CHU Poitiers, Université de Poitiers, Poitiers, France.
authors.forename: Guy
authors.initials: G
authors.lastname: Touchard
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology / Q000276:immunology / Q000473:pathology; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000682:Amyloid / Q000276:immunology; D000686:Amyloidosis / Q000209:etiology / Q000276:immunology / Q000473:pathology; D000911:Antibodies, Monoclonal / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology*; D015331:Cohort Studies; D005260:Female; D015432:Glomerulonephritis, Membranoproliferative / Q000209:etiology / Q000276:immunology / Q000473:pathology; D006801:Humans; D007075:Immunoglobulin M / Q000276:immunology*; D007674:Kidney Diseases / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D007680:Kidney Neoplasms / Q000150:complications* / Q000276:immunology / Q000473:pathology; D016393:Lymphoma, B-Cell / Q000150:complications* / Q000276:immunology / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009395:Nephritis, Interstitial / Q000209:etiology / Q000276:immunology / Q000473:pathology; D009404:Nephrotic Syndrome / Q000209:etiology / Q000276:immunology / Q000473:pathology; D010265:Paraproteinemias / Q000150:complications* / Q000276:immunology / Q000473:pathology; D012189:Retrospective Studies; D008258:Waldenstrom Macroglobulinemia / Q000150:complications* / Q000276:immunology / Q000473:pathology
chemical_list: D000682:Amyloid; D000911:Antibodies, Monoclonal; D007075:Immunoglobulin M; C518669:amyloid L, human
keywords: B-cell lymphoproliferative disorder; Fanconi syndrome; IgM; Monoclonal gammopathy; Waldenström magroglobulinemia; amyloidosis; cast nephropathy; kidney biopsy; membranoproliferative glomerulonephritis; renal response; tubular disorder