American Journal of Kidney Diseases

Use of Bortezomib in Heavy-Chain Deposition Disease: A Report of 3 Cases

Published:March 07, 2014DOI:
      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.

      Index Words

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to American Journal of Kidney Diseases
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Buxbaum J.
        • Gallo G.
        Nonamyloidotic monoclonal immunoglobulin deposition disease. Light-chain, heavy-chain, and light- and heavy-chain deposition diseases.
        Hematol Oncol Clin North Am. 1999; 13: 1235-1248
        • Gallo G.
        • Picken M.
        • Buxbaum J.
        • Frangione B.
        The spectrum of monoclonal immunoglobulin deposition disease associated with immunocytic dyscrasias.
        Semin Hematol. 1989; 26: 234-245
        • Aucouturier P.
        • Khamlichi A.A.
        • Touchard G.
        • et al.
        Brief report: heavy-chain deposition disease.
        N Engl J Med. 1993; 329: 1389-1393
        • Soma J.
        • Sato K.
        • Sakuma T.
        • et al.
        Immunoglobulin gamma3-heavy-chain deposition disease: report of a case and relationship with hypocomplementemia.
        Am J Kidney Dis. 2004; 43: E10-E16
        • Alexander M.P.
        • Nasr S.H.
        • Watson D.C.
        • Mendez G.P.
        • Rennke H.G.
        Renal crescentic alpha heavy chain deposition disease: a report of 3 cases and review of the literature.
        Am J Kidney Dis. 2011; 58: 621-625
        • Kambham N.
        • Markowitz G.S.
        • Appel G.B.
        • Kleiner M.J.
        • Aucouturier P.
        • D'Agati V.
        Heavy chain deposition disease: the disease spectrum.
        Am J Kidney Dis. 1999; 33: 954-962
        • Lin J.
        • Markowitz G.S.
        • Valeri A.M.
        • et al.
        Renal monoclonal immunoglobulin deposition disease: the disease spectrum.
        J Am Soc Nephrol. 2001; 12: 1482-1492
        • Masai R.
        • Wakui H.
        • Togashi M.
        • et al.
        Clinicopathological features and prognosis in immunoglobulin light and heavy chain deposition disease.
        Clin Nephrol. 2009; 71: 9-20
        • Nasr S.H.
        • Valeri A.M.
        • Cornell L.D.
        • et al.
        Renal monoclonal immunoglobulin deposition disease: a report of 64 patients from a single institution.
        Clin J Am Soc Nephrol. 2012; 7: 231-239
        • Oe Y.
        • Nakaya I.
        • Yahata M.
        • Sakuma T.
        • Sato H.
        • Soma J.
        A case of gamma1-heavy chain deposition disease successfully treated with melphalan and prednisolone therapy.
        Intern Med. 2010; 49: 1411-1415
        • Moreau P.
        The future of therapy for relapsed/refractory multiple myeloma: emerging agents and novel treatment strategies.
        Semin Hematol. 2012; 49: S33-S46
        • Richardson P.G.
        • Delforge M.
        • Beksac M.
        • et al.
        Management of treatment-emergent peripheral neuropathy in multiple myeloma.
        Leukemia. 2012; 26: 595-608
        • Richardson P.G.
        • Xie W.
        • Mitsiades C.
        • et al.
        Single-agent bortezomib in previously untreated multiple myeloma: efficacy, characterization of peripheral neuropathy, and molecular correlations with response and neuropathy.
        J Clin Oncol. 2009; 27: 3518-3525