Heavy chain deposition disease: An overview

Yuji Oe, Jun Soma, Hiroshi Sato, Sadayoshi Ito

Research output: Contribution to journalReview articlepeer-review

10 Citations (Scopus)

Abstract

Heavy chain deposition disease (HCDD) is one of three entities of monoclonal immunoglobulin deposition disease, characterized histopathologically by the presence of nodular glomerulosclerosis and glomerular and tubular deposition of monoclonal heavy chains without associated light chains. Although HCDD is an extremely rare disease, >30 cases have been reported to date in the literature. Of these cases, only three cases have been reported in Japan. The majority of the patients presents with nephrotic syndrome, hematuria, and hypertension, and develop progressive renal failure with or without the complication of multiple myeloma. Some cases have been treated successfully using chemotherapy. Because of its rarity, a thorough understanding of HCDD is essential for both accurate diagnosis and adequate subsequent treatment.

Original languageEnglish
Pages (from-to)771-778
Number of pages8
JournalClinical and experimental nephrology
Volume17
Issue number6
DOIs
Publication statusPublished - 2013 Dec

Keywords

  • Chemotherapy
  • Hematological disorder
  • Monoclonal immunoglobulin deposition disease
  • Nephrotic syndrome
  • Nodular glomerulosclerosis

ASJC Scopus subject areas

  • Physiology
  • Nephrology
  • Physiology (medical)

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