title: IgD heavy-chain deposition disease: detection by laser microdissection and mass spectrometry.
abstract: Monoclonal Ig deposition disease (MIDD) is a rare complication of monoclonal gammopathy characterized by deposition of monoclonal Ig light chains and/or heavy chains along the glomerular and tubular basement membranes. Here, we describe a unique case of IgD deposition disease. IgD deposition is difficult to diagnose, because routine immunofluorescence does not detect IgD. A 77-year-old man presented with proteinuria and renal failure, and kidney biopsy analysis showed a nodular sclerosing GN with extensive focal global glomerulosclerosis, tubular atrophy, and interstitial fibrosis. Immunofluorescence was negative for Ig deposits, although electron microscopy showed deposits in the glomeruli and along tubular basement membranes. Laser microdissection of glomeruli and mass spectrometry of extracted peptides showed a large spectra number for IgD, and immunohistochemistry showed intense glomerular and tubular staining for IgD. Together, these findings are consistent with IgD deposition disease. Bone marrow biopsy analysis showed 5% plasma cells, which stained for IgD. The patient was treated with bortezomib and dexamethasone, which resulted in improvement of hematologic parameters but no improvement of renal function. The diagnosis of IgD deposition disease underscores the value of laser microdissection and mass spectrometry in further evaluating renal biopsies when routine assessment fails to reach an accurate diagnosis.
authors.affiliation: Departments of Pathology and Cytology.
authors.forename: Virginie
authors.initials: V
authors.lastname: Royal
authors.affiliation: Department of Laboratory Medicine and Pathology and.
authors.forename: Patrick
authors.initials: P
authors.lastname: Quint
authors.affiliation: Nephrology, and.
authors.forename: Martine
authors.initials: M
authors.lastname: Leblanc
authors.affiliation: Hematology-Oncology, Maisonneuve-Rosemont Hospital, Montreal, Canada; and.
authors.forename: Richard
authors.initials: R
authors.lastname: LeBlanc
authors.affiliation: Department of Laboratory Medicine and Pathology and.
authors.forename: Garrett F
authors.initials: GF
authors.lastname: Duncanson
authors.affiliation: Department of Laboratory Medicine and Pathology and.
authors.forename: Robert L
authors.initials: RL
authors.lastname: Perrizo
authors.affiliation: Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota.
authors.forename: Fernando C
authors.initials: FC
authors.lastname: Fervenza
authors.affiliation: Department of Laboratory Medicine and Pathology and.
authors.forename: Paul
authors.initials: P
authors.lastname: Kurtin
authors.affiliation: Department of Laboratory Medicine and Pathology and sethi.sanjeev@mayo.edu.
authors.forename: Sanjeev
authors.initials: S
authors.lastname: Sethi
mesh_terms: D000368:Aged; D001706:Biopsy; D006362:Heavy Chain Disease / Q000175:diagnosis*; D006801:Humans; D007138:Immunoglobulin delta-Chains; D007668:Kidney / Q000473:pathology; D007674:Kidney Diseases / Q000175:diagnosis* / Q000276:immunology; D061346:Laser Capture Microdissection; D008297:Male; D013058:Mass Spectrometry
chemical_list: D007138:Immunoglobulin delta-Chains
keywords: immunology and pathology; multiple myeloma; renal pathology