Heavy chain deposition disease: an overview.

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.

journal_name

Clin Exp Nephrol

authors

Oe Y,Soma J,Sato H,Ito S

doi

10.1007/s10157-013-0812-x

subject

Has Abstract

pub_date

2013-12-01 00:00:00

pages

771-8

issue

6

eissn

1342-1751

issn

1437-7799

journal_volume

17

pub_type

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