American Journal of Kidney Diseases

Recurrent Light Chain Proximal Tubulopathy in a Kidney Allograft

      We describe a rare case of light chain proximal tubulopathy developing in a kidney transplant 12 months following transplantation. The patient was known to have a monoclonal gammopathy of undetermined significance (MGUS) for more than 15 years. A kidney biopsy done to determine the cause of decline in kidney transplant function showed light chain proximal tubulopathy characterized by numerous eosinophilic and fuchsinophilic granules in proximal tubular epithelial cells, which stained for κ light chains on pronase-based immunofluorescence studies. Electron microscopy confirmed the diagnosis and showed numerous amorphous and geometrically shaped inclusions in proximal tubular epithelial cells. Evaluation of free light chains revealed markedly elevated κ light chains and bone marrow biopsy showed 5% to 10% κ light chain–restricted plasma cells. Retrospective evaluation of the native kidney biopsy performed 15 years earlier also showed numerous fuchsinophilic granules in proximal tubules that stained brightly for κ light chains on pronase-based immunofluorescence studies. The patient was treated with a regimen of bortezomib and dexamethasone with good partial hematologic response and improvement of kidney function. To summarize, we describe a case of recurrent light chain proximal tubulopathy in the transplant, which is an unusual but important cause of decreased kidney function in the setting of a monoclonal gammopathy.

      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


        • Larsen C.P.
        • Bell J.M.
        • Harris A.A.
        • et al.
        The morphologic spectrum and clinical significance of light chain proximal tubulopathy with and without crystal formation.
        Mod Pathol. 2011; 24: 1462-1469
        • Stokes M.B.
        • Valeri A.M.
        • Herlitz L.
        • et al.
        Light chain proximal tubulopathy: clinical and pathologic characteristics in the modern treatment era.
        J Am Soc Nephrol. 2016; 27: 1555-1565
        • Nasr S.H.
        • Valeri A.M.
        • Sethi S.
        • et al.
        Clinicopathologic correlations in multiple myeloma: a case series of 190 patients with kidney biopsies.
        Am J Kidney Dis. 2012; 59: 786-794
        • Drieux F.
        • Loron M.C.
        • Francois A.
        • et al.
        Light chain deposition disease and proximal tubulopathy in two successive kidney allografts.
        Clin Nephrol. 2015; 83: 351-356
        • Batal I.
        • Schlossman R.L.
        • Safa K.
        • Rennke H.G.
        • Campenot E.S.
        Anemia, fatigue, and late-failing kidney transplant.
        Am J Kidney Dis. 2014; 64: A16-A19
        • Markowitz G.S.
        Dysproteinemia and the kidney.
        Adv Anat Pathol. 2004; 11: 49-63
        • Sethi S.
        • Rajkumar S.V.
        Monoclonal gammopathy–associated proliferative glomerulonephritis.
        Mayo Clin Proc. 2013; 88: 1284-1293
        • Bridoux F.
        • Leung N.
        • Hutchison C.A.
        • et al.
        Diagnosis of monoclonal gammopathy of renal significance.
        Kidney Int. 2015; 87: 698-711
        • Sethi S.
        • Fervenza F.C.
        • Rajkumar S.V.
        Spectrum of manifestations of monoclonal gammopathy-associated renal lesions.
        Curr Opin Nephrol Hypertens. 2016; 25: 127-137
        • Leboulleux M.
        • Lelongt B.
        • Mougenot B.
        • et al.
        Protease resistance and binding of Ig light chains in myeloma-associated tubulopathies.
        Kidney Int. 1995; 48: 72-79
        • Kapur U.
        • Barton K.
        • Fresco R.
        • et al.
        Expanding the pathologic spectrum of immunoglobulin light chain proximal tubulopathy.
        Arch Pathol Lab Med. 2007; 131: 1368-1372
        • Nasr S.H.
        • Galgano S.J.
        • Markowitz G.S.
        • et al.
        Immunofluorescence on pronase-digested paraffin sections: a valuable salvage technique for renal biopsies.
        Kidney Int. 2006; 70: 2148-2151
        • Messiaen T.
        • Deret S.
        • Mougenot B.
        • et al.
        Adult Fanconi syndrome secondary to light chain gammopathy. Clinicopathologic heterogeneity and unusual features in 11 patients.
        Medicine. 2000; 79: 135-154
        • Lorenz E.C.
        • Sethi S.
        • Leung N.
        • et al.
        Recurrent membranoproliferative glomerulonephritis after kidney transplantation.
        Kidney Int. 2010; 77: 721-728