Advertisement

Irish Journal of Medical Science

, Volume 183, Issue 2, pp 319–321 | Cite as

A case of renal γ2 heavy-chain deposition disease accompanied by rapid progressive renal failure

  • G.-T. Chen
  • X.-H. Liao
  • R.-Y. Yan
  • Y. Li
  • L. ZhangEmail author
Brief Report

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.

Keywords

Heavy-chain deposition disease Nodular glomerulosclerosis Renal failure 

Notes

Conflict of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

References

  1. 1.
    Lin J, Markowitz GS, Valeri AM et al (2001) Renal monoclonal immunoglobulin deposition disease: the disease spectrum. J Am Soc Nephrol 12:1482–1492. doi: 1046-6673/1207-1482 PubMedGoogle Scholar
  2. 2.
    Alexander MP, Nasr SH, Watson DC et al (2011) Renal crescentic alpha heavy chain deposition disease: a report of 3 cases and review of the literature. Am J Kidney Dis 58:621–625. doi: 10.1053/j.ajkd.2011.05.022 PubMedCrossRefGoogle Scholar
  3. 3.
    Aucouturier P, Khamlichi AA, Touchard G et al (1993) Brief report: heavy-chain deposition disease. N Engl J Med 329:1389–1393. doi: 10.1056/NEJM199311043291905 PubMedCrossRefGoogle Scholar
  4. 4.
    Soma J, Sato K, Sakuma T et al (2004) Immunoglobulin γ3-heavy-chain deposition disease: report of a case and relationship with hypocomplementemia. Am J Kidney Dis 43:E10–E16. doi: 10.1053/j.ajkd.2003.09.024 PubMedCrossRefGoogle Scholar
  5. 5.
    Cheng IK, Ho SK, Chan DT et al (1996) Crescentic nodular glomerulosclerosis secondary to truncated immunoglobulin alpha heavy chain deposition. Am J Kidney Dis 28:283–288. doi: 10.1016/S0272-6386(96)90315-7 PubMedCrossRefGoogle Scholar
  6. 6.
    Moulin B, Deret S, Mariette X et al (1999) Nodular glomerulosclerosis with deposition of monoclonal immunoglobulin heavy chains lacking C(H)1. J Am Soc Nephrol 10:519–528. doi: 1046-6673/1003-0519$03.00/0 Google Scholar
  7. 7.
    Vedder AC, Weening JJ, Krediet RT (2004) Intracapillary proliferative glomerulonephritis due to heavy chain deposition disease. Nephrol Dial Transplant 19:1302–1304. doi: 10.1093/ndt/gfg575 PubMedCrossRefGoogle Scholar
  8. 8.
    Tan S, Pon K, Bargman J et al (2003) Generalized cutis laxa associated with heavy chain deposition disease. J Cutan Med Surg 7:390–394. doi: 10.1007/s10227-002-0128-z PubMedCrossRefGoogle Scholar
  9. 9.
    Harrington CR, Beswick TC, Susa JS et al (2008) Acquired cutis laxa associated with heavy chain deposition disease. J Am Acad Dermatol 59:S99–S101. doi: 10.1016/j.jaad.2008.05.007 PubMedCrossRefGoogle Scholar
  10. 10.
    Oe Y, Nakaya I, Yahata M et al (2010) A case of γ1-heavy chain deposition disease successfully treated with melphalan and prednisolone therapy. Intern Med 49:1411–1415. doi: 10.2169/internalmedicine.49.3499 PubMedCrossRefGoogle Scholar
  11. 11.
    Soma J, Tsuchiya Y, Sakuma T et al (2008) Clinical remission and histopathological resolution of nodular lesions in a patient with 3 heavy-chain deposition disease. Clin Nephrol 69:383–386PubMedCrossRefGoogle Scholar
  12. 12.
    Herzenberg AM, Kiaii M, Magil AB (2000) Heavy chain deposition disease: recurrence in a renal transplant and report of IgG(2) subtype. Am J Kidney Dis 35:E25. doi: 10.1053/kd.2000.6429 PubMedCrossRefGoogle Scholar

Copyright information

© Royal Academy of Medicine in Ireland 2013

Authors and Affiliations

  • G.-T. Chen
    • 1
  • X.-H. Liao
    • 1
  • R.-Y. Yan
    • 1
  • Y. Li
    • 1
  • L. Zhang
    • 1
    Email author
  1. 1.Department of NephrologyThe Second Affiliated Hospital, Chongqing Medical UniversityChongqingChina

Personalised recommendations