title: 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.
authors: 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.
authors: Yuji
authors: Y
authors: Oe
authors: Jun
authors: J
authors: Soma
authors: Hiroshi
authors: H
authors: Sato
authors: Sadayoshi
authors: S
authors: Ito
mesh_terms: D005260:Female; D006362:Heavy Chain Disease / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000737:chemistry; D007668:Kidney / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000150:complications
chemical_list: D007143:Immunoglobulin Heavy Chains
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