A case of γ 1-heavy chain deposition disease successfully treated with melphalan and prednisolone therapy

Yuji Oe, Izaya Nakaya, Mayumi Yahata, Tsutomu Sakuma, Hiroshi Sato, Jun Soma

Research output: Contribution to journalArticlepeer-review

12 Citations (Scopus)

Abstract

Heavy chain deposition disease (HCDD) is characterized by glomerular and tubular deposition of nonamyloidotic monoclonal heavy chains without associated light chains. We describe a case of γ1-HCDD who presented with nephrotic syndrome, microhematuria, and hypocomplementemia. Renal biopsy showed lobular and nodular glomerulosclerosis along with IgG and C3 deposition. Electron microscopy revealed electrondense deposits on the glomerular and tubular basement membranes and mesangium. Congo red staining was negative. Staining was positive for IgG1 on glomerular and tubular basement membranes but negative for IgG2-4, κ and λ light chains. Staining for the constant heavy chain domains showed the deletion of the first constant heavy chain domain. Thus, we diagnosed γ1-HCDD. She was considered to be early-stage HCDD because proteinuria and hematuria were not observed before the nephrotic syndrome onset. Melphalan and prednisolone (MP) therapy reduced proteinuria as well as improved renal function and complement levels. Although renal prognosis of HCDD is poor, aggressive chemotherapy with MP may be effective in earlystage HCDD patients.

Original languageEnglish
Pages (from-to)1411-1415
Number of pages5
JournalInternal Medicine
Volume49
Issue number14
DOIs
Publication statusPublished - 2010

Keywords

  • Heavy chain deposition disease
  • Melphalan
  • Prednisolone
  • Remission

ASJC Scopus subject areas

  • Internal Medicine

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