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Heavy chain deposition disease: an overview

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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.

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Conflict of interest

The authors declare that they have no relevant financial interests.

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Authors and Affiliations

  1. Division of Nephrology, Endocrinology, and Vascular Medicine, Graduate School of Medicine, Tohoku University, 1-1 Seiryo-cho, Sendai, 980-8574, Japan

    Yuji Oe & Sadayoshi Ito

  2. Department of Nephrology, Iwate Prefectural Central Hospital, Morioka, Japan

    Jun Soma

  3. Department of Clinical Pharmacology and Therapeutics, Graduate School of Pharmaceutical Sciences and Faculty of Pharmaceutical Sciences, Tohoku University, Sendai, Japan

    Hiroshi Sato

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  1. Yuji Oe
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  2. Jun Soma
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  4. Sadayoshi Ito
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Correspondence to Yuji Oe.

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Oe, Y., Soma, J., Sato, H. et al. Heavy chain deposition disease: an overview. Clin Exp Nephrol 17, 771–778 (2013). https://doi.org/10.1007/s10157-013-0812-x

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  • DOI: https://doi.org/10.1007/s10157-013-0812-x

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