Electrolyte Blood Press.  2016 Jun;14(1):5-10. 10.5049/EBP.2016.14.1.5.

A Rare Case of Polyneuropathy and Monoclonalgammopathy with Recurrent Acute Kidney Injury

• Kim EJ
• Shin DH
• Jeon HJ
• Rhee SY
• Nam ES
• Park JY
• Oh J

• KMID: 2328424
• DOI: http://doi.org/10.5049/EBP.2016.14.1.5

Abstract

POEMS syndrome is a rare paraneoplastic syndrome and there are few reports of polyneuropathy and monoclonal gammopathy associated with kidney dysfunction. Here, we report a case of POEMS syndrome with recurrent acute kidney injury (AKI). A 52-year-old man presented with bilateral aggravating paresthesia and latermotor weakness of the lower extremities accompanied by repeated elevation of serum creatinine. The patient was finally diagnosed with POEMS syndrome on the basis of fulfilling the two mandatory major criteria (polyneuropathy and monoclonal gammopathy), one other major criterion (sclerotic bone lesion), and several minor criteria. A renal biopsy was performed to clarify the cause of AKI and showed membranoproliferative glomerulonephritis-like lesions with mesangiolysis and endothelial cell injury. This case illustrates that renal manifestations, not included in the diagnostic criteria for POEMS, can be apparent before various other systemic symptoms.

Keyword

Acute kidney injury; Monoclonal gammopathy; POEMS syndrome; Polyneuropathy

MeSH Terms

Acute Kidney Injury*
Biopsy
Creatinine
Endothelial Cells
Humans
Kidney
Lower Extremity
Middle Aged
Paraneoplastic Syndromes
Paraproteinemias
Paresthesia
POEMS Syndrome
Polyneuropathies*
Creatinine

Figure

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  Fig. 1 Renal pathologic findings. (A) The results showed that most glomeruli appeared to increase in size and cellularity with moderate endocapillary proliferation (Hematoxylin-eosin stain, ×200). (B) Membranoproliferative like glomerulopathy with clear double contours of glomerular capillary walls (arrow) is high-lighted with Jone's methenamine silver stain reagent at a higher magnification (Jone's methenamine silver stain, ×200). (C) Electron microscopy clearly revealed most glomerular capillary loops are compromised with diffuse duplication of capillary walls and subendothelial widening with flocculent materials and mesangial interposition (Electron micrograph, ×3,000). (D) There was mesangiolysis with the development of capillary microaneurysm(arrowheads), but no electron dense deposits at any locations. Visceral epithelial cells showed hyperplasia and focal effacement of foot process about 20% (Electron micrograph, ×3,000).

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  Fig. 2 Iliac crest bone marrow biopsy specimen. (A) Immunohistochemical staining for κ light chain. Only small number of plasma cells are positive for immunoglobulin κ light chain (Immunohistochemical stain, ×100). (B) Immunohistochemical staining for λ light chain. Majority of plasma cells are positive for λ light chain (Immunohistochemical stain, ×100).

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  Fig. 3 Chest computed tomography (CT). Osteosclerotic lesion (arrows) in the posterior column of T6 is shown in the axial view (A) and coronal view (B) of the chest CT.

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