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Images in Nephrology
(Section Editor: G. H. Neild)
Multiple myeloma in a kidney transplanted patient primarily
diagnosed with monoclonal gammopathy of unknown significance
(MGUS)-related nephropathy
Marsela Resuli1,2, Finn Thomsen Nielsen1,2, Peter Gimsing3, Claus B. Andersen4and Martin Egfjord2
1
Department of Internal Medicine, Bornholm Hospital, Capital Region, Denmark,
2
Department of Nephrology, Rigshospitalet, University
of Copenhagen, Copenhagen, Denmark,
3
Department of Haematology, Rigshospitalet, University of Copenhagen, Copenhagen,
Denmark and
4
Department of Pathology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark
Correspondence and offprint requests to: Marsela Resuli; E-mail: marselaresuli@gmail.com
Keywords: kidney transplantation; light-chain deposition disease; monoclonal gammopathy of unknown significance; multiple myeloma
Case
A 36-year-old female was referred with renal impairment,
creatinine clearance 0.4 mL/s and proteinuria 4.7 g/day.
Physical examination was unremarkable. Biochemistry
showed normochromic normocytic anaemia, slight hypoal-
buminaemia and hypercalcaemia. Anti-neutrophil cyto-
plasmic antibodies, anti-nuclear antibodies, anti-glomerular
basement membrane, anti-phospholipid antibodies, and
viral screening were negative. IgG-kappa M-protein was
found in plasma (4.8 g/L) and urine (<0.02 g/L), while plasma
immunoglobulins and liver enzymes were normal. Bone
marrow, skeleton scintigraphy and MR scan were normal.
An ultrasound showed normal-sized kidneys. Kidney
biopsy presented focal interstitial nephritis, light grade
Fig. 1. Native kidney biopsy showing a glomerulus with a slight
homogenous thickening of capillary and arteriolar walls, minimal tubular
atrophy and a discrete interstitial fibrosis (PAS staining, magnification ×50).
Fig. 2. Eight years later: (a) Kidney-graft biopsy 4 years after
transplantation demonstrating nodular glomerular sclerosis, atrophic
tubuli and sclerotic arterioles similar to the end-stage native kidney (PAS,
magnification ×100). (b) Immunostaining was strongly positive for kappa
light chains in the expanded mesangial regions and arterioles.
© The Author 2013. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.
For permissions, please email: journals.permissions@oup.com.
Clin Kidney J (2013) 6: 445–446
doi: 10.1093/ckj/sft060
Advance Access publication 2 July 2013
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interstitial fibrosis but no glomerulonephritis, vasculitis or
tubular damage, and negative Congo staining (Figure 1).
The diagnoses were monoclonal gammopathy of
unknown significance (MGUS) and idiopathic interstitial
nephritis. Despite corticosteroid treatment, renal insuffi-
ciency progressed and, after 17 months, haemodialysis
was initiated.
Four years later, the patient received a kidney transplan-
tation with a standard immunosuppressive regimen: thy-
moglobulin induction, prednisolone, mycofenolat mofetil
and cyclosporine. The optimal plasma creatinine after
transplantation was 0.112 mmol/L. Unfortunately, 4 years
after transplantation renal function declined to a plasma
creatinine of 0.219 mmol/L. A kidney-graft biopsy at this
point showed global nodular glomerulosclerosis and
deposition of kappa light chains within glomeruli, vessels,
and tubular basal membranes, plus C3c in the vessels,
characteristic of light-chain deposition disease (LCDD) with
no rejection signs or amyloid deposits (Figure 2a and b).
P-IgG kappa M-protein was 0.86 g/L and Bence-Jones
proteins were present in the urine. A bone marrow re-
examination presented clonal plasma cell infiltration of
kappa type with a kappa/lambda ratio of 19.8.
Presenting M-protein, clonal bone marrow infiltration
and renal graft LCDD changes, the patient was diagnosed
with multiple myeloma [1]. Reevaluation of native kidney
biopsies revealed a weak-positive reaction for kappa light
chains in glomerular nodules, suggesting that LCDD might
have contributed to the renal insufficiency. LCDD has a
high recurrence risk and poor graft function prognosis
after kidney transplantation [2, 3]. Diagnosing LCDD might
be difficult in the early stage. Therefore, patients with a
pre-existing MGUS should be carefully evaluated and fol-
lowed prior to and after kidney transplantation [2, 3].
Conflict of interest statement. None declared.
References
1. International Myeloma Working Group. Criteria for the classifi-
cation of monoclonal gammopathies, multiple myeloma and
related disorders: a report of the International Myeloma
Working Group. Br J Haematol 2003; 121: 749–757
2. Ponticelli C, Moroni G, Glassock R. Recurrence of secondary glo-
merular disease after renal transplantation. Clin J Am Soc
Nephrol 2011; 6: 1214–1221
3. Leung N, Lager DJ, Gertz MA et al. Long-term outcome of renal
transplantation in light chain deposition disease. Am J Kidney
Dis 2004; 43: 147–153
Received for publication: 10.4.13; Accepted in revised form: 3.5.13
446 M. Resuli et al.
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