title: A case of renal γ2 heavy-chain deposition disease accompanied by rapid progressive renal failure.
abstract: BACKGROUND
  The recently described heavy-chain deposition disease (HCDD) is a comparatively rare monoclonal immunoglobulin disorder characterized histopathologically by glomerular and tubular basement membrane deposition of nonamyloidotic monoclonal heavy chains without associated light chains.
  
  
  METHODS
  We have described a case of γ2-HCDD presenting with proteinuria, microhematuria, severe hypertension, and rapidly progressive renal failure, which serum creatinine level was only 1.52 mg/dL in early stage HCDD, and the clinicopathologic features of this case have been compared with other reported cases of γ2-HCDD.
  
  
  RESULTS
  Renal biopsy disclosed nodular sclerosing glomerulopathy. Immunofluorescence analysis revealed IgG2 (2+) heavy chain and C3 (+) in the mesangium and along the capillary walls and tubular basement membranes without IgA, IgM, κ and λ light chains. Electron microscopy revealed electron-dense deposits along the glomerular and tubular basement membranes as well as in the mesangium. Moreover, regardless of therapy, the condition of the patient progressively deteriorated, with less than 3 months of renal survival.
  
  
  CONCLUSIONS
  Rapid progressive renal failure was a common feature in both cases of γ2-HCDD. We propose that a possible link exists between prognosis of renal HCDD and the subclass of heavy chain deposited in the kidney.
authors: Department of Nephrology, The Second Affiliated Hospital, Chongqing Medical University, Chongqing, 400010, China.
authors: G-T
authors: GT
authors: Chen
authors: X-H
authors: XH
authors: Liao
authors: R-Y
authors: RY
authors: Yan
authors: Y
authors: Y
authors: Li
authors: L
authors: L
authors: Zhang
mesh_terms: D001706:Biopsy; D003176:Complement C3 / Q000032:analysis; D018450:Disease Progression; D006362:Heavy Chain Disease / Q000150:complications / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007143:Immunoglobulin Heavy Chains / Q000032:analysis; D007668:Kidney / Q000473:pathology; D007678:Kidney Glomerulus; D008297:Male; D008875:Middle Aged; D051437:Renal Insufficiency / Q000276:immunology / Q000473:pathology*
chemical_list: D003176:Complement C3; D007074:Immunoglobulin G; D007143:Immunoglobulin Heavy Chains
references: Intern Med. 2010;49(14):1411-5
references: 20647658
references: Nephrol Dial Transplant. 2004 May;19(5):1302-4
references: 15102968
references: N Engl J Med. 1993 Nov 4;329(19):1389-93
references: 8413435
references: J Am Soc Nephrol. 2001 Jul;12(7):1482-1492
references: 11423577
references: Am J Kidney Dis. 2004 Jan;43(1):E10-6
references: 14712467
references: J Am Acad Dermatol. 2008 Nov;59(5 Suppl):S99-101
references: 19119136
references: Clin Nephrol. 2008 May;69(5):383-6
references: 18538103
references: Am J Kidney Dis. 2011 Oct;58(4):621-5
references: 21944962
references: J Am Soc Nephrol. 1999 Mar;10(3):519-28
references: 10073602
references: J Cutan Med Surg. 2003 Sep-Oct;7(5):390-4
references: 14973644
references: Am J Kidney Dis. 2000 May;35(5):E25
references: 10793054
references: Am J Kidney Dis. 1996 Aug;28(2):283-8
references: 8768927