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Clin Exp Nephrol. 2013 Dec;17(6):771-8. doi: 10.1007/s10157-013-0812-x. Epub 2013 May 8.

Heavy chain deposition disease: an overview.

Author information

1
Division of Nephrology, Endocrinology, and Vascular Medicine, Graduate School of Medicine, Tohoku University, 1-1 Seiryo-cho, Sendai, 980-8574, Japan, yuji-oe@med.tohoku.ac.jp.

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.

PMID:
23652830
DOI:
10.1007/s10157-013-0812-x
[Indexed for MEDLINE]

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