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.

METHOD:

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.

CONCLUSION:

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: G-T Chen, X-H Liao, R-Y Yan, Y Li, L Zhang
• Journal: Irish journal of medical science (Ir J Med Sci) Vol. 183 Issue 2 Pg. 319-21 (Jun 2014) ISSN: 1863-4362 [Electronic] Ireland
• PMID: 23963539 (Publication Type: Case Reports, Journal Article)

Chemical References

• Complement C3
• Immunoglobulin G
• Immunoglobulin Heavy Chains

Topics

• Biopsy
• Complement C3 (analysis)
• Disease Progression
• Heavy Chain Disease (complications, immunology, pathology)
• Humans
• Immunoglobulin G (analysis)
• Immunoglobulin Heavy Chains (analysis)
• Kidney (pathology)
• Kidney Glomerulus
• Male
• Middle Aged
• Renal Insufficiency (immunology, pathology)