Use of bortezomib in heavy-chain deposition disease

A report of 3 cases

Kinjal Patel, John J. Dillon, Nelson Leung, Andrew S. Bomback, Gerald B. Appel, Vivette D'Agati, Pietro A. Canetta

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Heavy-chain deposition disease (HCDD) is a rare complication of plasma cell dyscrasia in which monoclonal heavy chains deposit in glomerular and tubular basement membranes of the kidney. Clinical and pathologic features of HCDD have been well described in case reports and series, but evidence supporting specific therapies is sparse. Historically, the disease has had a poor prognosis, intensifying the need to clarify optimal treatments. We describe 3 cases of HCDD with biopsy-proven glomerular involvement, severe nephrotic syndrome, and decline in kidney function that were treated successfully with bortezomib, a proteasome inhibitor. None of these patients had multiple myeloma. In all cases, bortezomib-based therapy resulted in sustained resolution of nephrotic syndrome and improvement in kidney function. All 3 patients developed peripheral neuropathy; otherwise, treatment was well tolerated. To our knowledge, this is the first description of the clinical effectiveness of bortezomib against HCDD.

Original languageEnglish (US)
Pages (from-to)123-127
Number of pages5
JournalAmerican Journal of Kidney Diseases
Volume64
Issue number1
DOIs
StatePublished - 2014

Fingerprint

Heavy Chain Disease
Nephrotic Syndrome
Kidney
Glomerular Basement Membrane
Paraproteinemias
Proteasome Inhibitors
Peripheral Nervous System Diseases
Therapeutics
Multiple Myeloma
Biopsy
Bortezomib

Keywords

  • bortezomib
  • Heavy-chain deposition disease
  • Index Words
  • monoclonal immunoglobulin deposition disease
  • nephrotic syndrome

ASJC Scopus subject areas

  • Nephrology

Cite this

Patel, K., Dillon, J. J., Leung, N., Bomback, A. S., Appel, G. B., D'Agati, V., & Canetta, P. A. (2014). Use of bortezomib in heavy-chain deposition disease: A report of 3 cases. American Journal of Kidney Diseases, 64(1), 123-127. https://doi.org/10.1053/j.ajkd.2014.01.425

Use of bortezomib in heavy-chain deposition disease : A report of 3 cases. / Patel, Kinjal; Dillon, John J.; Leung, Nelson; Bomback, Andrew S.; Appel, Gerald B.; D'Agati, Vivette; Canetta, Pietro A.

In: American Journal of Kidney Diseases, Vol. 64, No. 1, 2014, p. 123-127.

Research output: Contribution to journalArticle

Patel, K, Dillon, JJ, Leung, N, Bomback, AS, Appel, GB, D'Agati, V & Canetta, PA 2014, 'Use of bortezomib in heavy-chain deposition disease: A report of 3 cases', American Journal of Kidney Diseases, vol. 64, no. 1, pp. 123-127. https://doi.org/10.1053/j.ajkd.2014.01.425
Patel, Kinjal ; Dillon, John J. ; Leung, Nelson ; Bomback, Andrew S. ; Appel, Gerald B. ; D'Agati, Vivette ; Canetta, Pietro A. / Use of bortezomib in heavy-chain deposition disease : A report of 3 cases. In: American Journal of Kidney Diseases. 2014 ; Vol. 64, No. 1. pp. 123-127.
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