title: Outcomes of patients with renal monoclonal immunoglobulin deposition disease.
abstract: Recent reports suggest that deep hematologic responses to chemotherapy are associated with improved renal outcomes in monoclonal immunoglobulin deposition disease (MIDD). Here we describe the long term outcomes and identify prognostic factors after first line treatment of the largest reported series of patients with MIDD. Between March 1992 and December 2014, 88 patients with MIDD were seen at Mayo Clinic, MN. Renal responses were defined using criteria used for light chain amyloidosis (AL) or those used by the IMWG. Sixty-one (69%) patients had a GFR < 30 mL/min/1.73 m2and 16 (18%) were on renal replacement therapy at diagnosis. The interval between albuminuria or elevation in creatinine and MIDD diagnosis was 12 months suggesting a delay in diagnosis. Thirty-seven patients (42%) had at least a hematologic CR/VGPR. Fifty-three (60%) received an autologous stem cell transplant (ASCT) or proteasome inhibitor (PI)-based treatments. Patients receiving ASCT or PI-based therapies were more likely to achieve at least a hematologic CR/VGPR compared to those receiving other therapies: 66% vs 2%, p < 0.0001. Patients that achieved a hematologic CR were more likely to achieve a renal response (53% vs 24%, p = 0.001). Five year overall and renal survival for the entire cohort was 67% and 57%, respectively. In multivariate analyses, a baseline GFR < 20 mL/min/1.73 m2and a renal response (using AL or IMWG criteria) were independently predictive of progression to dialysis. This study confirms that deep hematologic responses, best achieved with ASCT or PI-based therapies, are a prerequisite to achieving renal responses. Am. J. Hematol. 91:1123-1128, 2016. © 2016 Wiley Periodicals, Inc.
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: Taxiarchis V
authors: TV
authors: Kourelis
authors: Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.
authors: Samih H
authors: SH
authors: Nasr
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: Angela
authors: A
authors: Dispenzieri
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: Shaji K
authors: SK
authors: Kumar
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: Morie A
authors: MA
authors: Gertz
authors: Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota.
authors: Fernando C
authors: FC
authors: Fervenza
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: Francis K
authors: FK
authors: Buadi
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: Martha Q
authors: MQ
authors: Lacy
authors: Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota.
authors: Stephen B
authors: SB
authors: Erickson
authors: Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota.
authors: Fernando G
authors: FG
authors: Cosio
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: Prashant
authors: P
authors: Kapoor
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: John A
authors: JA
authors: Lust
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: Suzanne R
authors: SR
authors: Hayman
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: Vincent
authors: V
authors: Rajkumar
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: Steven R
authors: SR
authors: Zeldenrust
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: Stephen J
authors: SJ
authors: Russell
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: David
authors: D
authors: Dingli
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: Yi
authors: Y
authors: Lin
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: Wilson
authors: W
authors: Gonsalves
authors: Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota.
authors: Elizabeth C
authors: EC
authors: Lorenz
authors: Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota.
authors: Ladan
authors: L
authors: Zand
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors: Robert A
authors: RA
authors: Kyle
authors: Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota. leung.nelson@mayo.edu.|Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota. leung.nelson@mayo.edu.
authors: Nelson
authors: N
authors: Leung
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000032:analysis*; D007674:Kidney Diseases / Q000401:mortality / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000401:mortality / Q000628:therapy*; D061988:Proteasome Inhibitors / Q000627:therapeutic use; D012189:Retrospective Studies; D033581:Stem Cell Transplantation / Q000379:methods; D016019:Survival Analysis; D014182:Transplantation, Autologous; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007147:Immunoglobulin Light Chains; D061988:Proteasome Inhibitors