A case of multiple myeloma presenting with uric acid kidney stones

Abstract

Kidney disease is common in patients with multiple myeloma. Patients often present with acute kidney failure most commonly the result of light chain cast nephropathy. Presentation can also be in the form of nephrotic syndrome associated with immunoglobulin light chain amyloidosis or monoclonal immunoglobulin deposition disease and tubulopathy as in acquired light chain Fanconi syndrome. We present a case of a 56-year-old male with multiple myeloma who presented with chronic kidney disease and uric acid stones. Chemical analysis of the nephrolithiasis showed it to be entirely composed of uric acid. Fractional excretion of uric acid was elevated at 35.9%. Kidney biopsy was consistent with light chain proximal tubulopathy. Bone marrow biopsy showed 30% kappa light chain–restricted plasma cells. Genetic sequencing identified the light chain to be from the Vk1-33 subgroup. The patient was treated with bortezomib, cyclophosphamide, and dexamethasone, with improvement of his kidney function. This case illustrates a rare presentation of multiple myeloma.

Keywords

Multiple myeloma monoclonal protein crystals light chains

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References

Stokes

Valeri

Herlitz

et al . Light chain proximal tubulopathy: clinical and pathologic characteristics in the modern treatment era. J Am Soc Nephrol 2015; 27: 1555–1565.

Sharma

Bonsib

Gokden

Light chain proximal tubulopathy: expanding the pathologic spectrum with deposition of crystalline-like inclusions in glomeruli and interstitium in addition to proximal tubules. Modern Pathol 2011; 24: 461a.

Larsen

Bell

Harris

et al . The morphologic spectrum and clinical significance of light chain proximal tubulopathy with and without crystal formation. Mod Pathol 2011; 24(11): 1462–1469.

Sirac

Bridoux

Carrion

et al . Role of the monoclonal kappa chain V domain and reversibility of renal damage in a transgenic model of acquired Fanconi syndrome. Blood 2006; 108(2): 536–543.

Luciani

Sirac

Terryn

et al . Impaired lysosomal function underlies monoclonal light chain-associated renal Fanconi syndrome. J Am Soc Nephrol 2016; 27: 2049–2061.

Brodie

Agrawal

Rahemtulla

et al . Multiple myeloma with bone marrow extracellular crystal deposition. J Clin Pathol 2007; 60(9): 1064–1065.

Bridoux

Sirac

Hugue

et al . Fanconi’s syndrome induced by a monoclonal Vkappa3 light chain in Waldenstrom’s macroglobulinemia. Am J Kidney Dis 2005; 45(4): 749–757.

Brochet

Lefranc

M-P

Giudicelli

IMGT/V-QUEST: the highly customized and integrated system for IG and TR standardized V-J and V-D-J sequence analysis. Nucleic Acids Res 2008; 36: W503–158.

Engle

Jr Wallis

. Multiple myeloma and the adult Fanconi syndrome. I. Report of a case with crystal-like deposits in the tumor cells and in the epithelial cells of the kidney. Am J Med 1957; 22(1): 5–23.

10.

Kapur

Barton

Fresco

et al . Expanding the pathologic spectrum of immunoglobulin light chain proximal tubulopathy. Arch Pathol Lab Med 2007; 131(9): 1368–1372.

11.

Vignon

Javaugue

Alexander

et al . Current anti-myeloma therapies in renal manifestations of monoclonal light chain-associated Fanconi syndrome: a retrospective series of 49 patients. Leukemia 2017; 31(1): 123–129.

12.

Weir

Schweitzer

FA.

Multiple myeloma presenting as recurrent ureteric obstruction. J R Soc Med 1996; 89(12): 707.

13.

Sirac

Bridoux

Essig

et al . Toward understanding renal Fanconi syndrome: step by step advances through experimental models. Contrib Nephrol 2011; 169: 247–261.

14.

Leung

Bridoux

Batuman

et al . The evaluation of monoclonal gammopathy of renal significance: a consensus report of the International Kidney and Monoclonal Gammopathy Research Group. Nat Rev Nephrol 2019; 15: 45–59.

15.

Leung

Bridoux

Hutchison

et al . Monoclonal gammopathy of renal significance: when MGUS is no longer undetermined or insignificant. Blood 2012; 120: 4292–4295.