title: Renal crescentic alpha heavy chain deposition disease: a report of 3 cases and review of the literature.
abstract: Heavy chain deposition disease (HCDD) is a comparatively recently described entity characterized by glomerular and tubular basement membrane deposition of monoclonal heavy chains without associated light chains. To our knowledge, review of the literature shows only 24 previously reported cases of HCDD with unequivocal evidence of monoclonal heavy chain deposition in the kidney using immunofluorescence microscopic and electron microscopic studies. The predominant heavy chain subtype was γ. There has been a single case of μ HCDD and 2 previously reported cases of α HCDD. In this report, we describe 3 additional cases of α HCDD, all with a crescentic pattern of injury and one of which was associated with cutis laxa. We compare their clinicopathologic features with all previously reported cases of HCDD.
authors.affiliation: Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA. alexander.mariam@mayo.edu
authors.forename: Mariam P
authors.initials: MP
authors.lastname: Alexander
authors.forename: Samih H
authors.initials: SH
authors.lastname: Nasr
authors.forename: Didie C
authors.initials: DC
authors.lastname: Watson
authors.forename: Gonzalo P
authors.initials: GP
authors.lastname: Méndez
authors.forename: Helmut G
authors.initials: HG
authors.lastname: Rennke
mesh_terms: D000328:Adult; D000368:Aged; D000740:Anemia / Q000209:etiology; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001897:Boronic Acids / Q000008:administration & dosage; D000069286:Bortezomib; D003483:Cutis Laxa / Q000209:etiology*; D003907:Dexamethasone / Q000008:administration & dosage; D003928:Diabetic Nephropathies / Q000209:etiology / Q000276:immunology*; D004921:Erythropoietin / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D006362:Heavy Chain Disease / Q000276:immunology* / Q000473:pathology; D006417:Hematuria / Q000209:etiology; D006801:Humans; D006977:Hypertension, Renal / Q000209:etiology; D007137:Immunoglobulin alpha-Chains / Q000032:analysis; D007142:Immunoglobulin gamma-Chains / Q000032:analysis; D007148:Immunoglobulin mu-Chains / Q000032:analysis; D007678:Kidney Glomerulus / Q000276:immunology / Q000473:pathology*; D008297:Male; D009101:Multiple Myeloma / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D010265:Paraproteinemias / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D011507:Proteinuria / Q000209:etiology; D011719:Pyrazines / Q000008:administration & dosage; D013792:Thalidomide / Q000008:administration & dosage; D014581:Urticaria / Q000209:etiology; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000209:etiology
chemical_list: D001897:Boronic Acids; D007137:Immunoglobulin alpha-Chains; D007142:Immunoglobulin gamma-Chains; D007148:Immunoglobulin mu-Chains; D011719:Pyrazines; D004921:Erythropoietin; D013792:Thalidomide; D000069286:Bortezomib; D003907:Dexamethasone