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Year : 2018  |  Volume : 14  |  Issue : 3  |  Page : 679-681

An atypical presentation of POEMS syndrome with IgG kappa type M protein and normal VEGF level: Case report and review of literature

1 Department of Research, Health East Care System, Saint Paul, Minnesota, 55102, USA
2 Department of Internal Medicine, Health East Care System, Saint Paul, Minnesota, 55102, USA

Date of Web Publication12-Jun-2018

Correspondence Address:
Dr. Murali Krishna Gurram
45, 10th Street West, Saint Paul, Minnesota 55102
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.172123

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 > Abstract 

POEMS syndrome is a multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. We present an atypical case of POEMS syndrome with subclinical polyneuropathy, splenomegaly, hypogonadism, and myeloma of immunoglobulin G kappa type. The typical osteosclerotic lesions and skin lesions were absent, and vascular endothelial growth factor (VEGF) level was normal. Our patient also had chronic kidney disease due to focal segmental glomerulosclerosis. The patient died due to multisystem complications nine months after presenting with acute on chronic renal failure. POEMS syndrome is an uncommon, potentially fatal paraneoplastic syndrome presenting with plasma cell neoplasm and multisystem involvement. Despite the normal VEGF level, our patient was diagnosed to have possible POEMS syndrome due to other clinical manifestations. It may be reasonable to expand the diagnostic criteria of POEMS syndrome if further atypical cases are reported in the future.

Keywords: IgG kappa light chain, POEMS syndrome, renal involvement

How to cite this article:
Pulivarthi S, Gurram MK. An atypical presentation of POEMS syndrome with IgG kappa type M protein and normal VEGF level: Case report and review of literature. J Can Res Ther 2018;14:679-81

How to cite this URL:
Pulivarthi S, Gurram MK. An atypical presentation of POEMS syndrome with IgG kappa type M protein and normal VEGF level: Case report and review of literature. J Can Res Ther [serial online] 2018 [cited 2020 Nov 25];14:679-81. Available from: https://www.cancerjournal.net/text.asp?2018/14/3/679/172123

 > Introduction Top

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome or Crow–Fukase syndrome is a rare paraneoplastic syndrome of unknown cause characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes.[1] The diagnosis of this syndrome is based on two mandatory major criteria, polyneuropathy and monoclonal plasmaproliferative disorder (almost always lambda) and one of three other major criteria, osteosclerotic lesions, elevated vascular endothelial growth factor (VEGF), and Castleman's disease.[1] We present an atypical case of multiple myeloma of immunoglobulin G (IgG) kappa type with end-organ involvement suggestive of possible POEMS syndrome.

 > Case Report Top

A 60-year-old Caucasian male with a history of chronic kidney disease due to biopsy proven focal segmental glomerulosclerosis (FSGS) presented with severe renal dysfunction and was started on hemodialysis due to poor response to cyclosporine and steroids. Two months later, he presented with abdominal swelling due to massive ascites. He does not smoke, drink alcohol, or use illicit drugs and he had three children. The patient denies any tingling or numbness in extremities. He appears emaciated with neither skin lesions nor lymphadenopathy on physical exam. Lab work showed hyperbilirubinemia, thrombocytopenia, anemia, and coagulopathy. Liver cirrhosis work-up was negative for hepatitis B or C infection, ceruloplasmin, anti-mitochondrial antibody, and anti-smooth muscle antibody. Serum ascites-albumin gradient was 1.3, which was consistent with cardiogenic origin of ascites. Liver biopsy done twice was negative for fibrosis, xerosis, cirrhosis, and steatosis. The patient had bilateral pleural effusion and mild pericardial effusion. Echocardiogram revealed a flail tricuspid leaflet with eccentric, severe tricuspid regurgitation, and normal left ventricular chamber size with 65% ejection fraction.

Serum immunoelectrophoresis showed an M protein spike of 2.1 g/dl identified as an IgG kappa type of monoclonal immunoglobulin. VEGF level was 80 pg/ml (normal 62–700 pg/ml) and interleukin-6 level was 174.4 (normal range <4 pg/ml). Bone marrow biopsy confirmed multiple myeloma with 20% monoclonal plasma cells. There was no evidence of amyloidosis on the fat pad aspirate. Electromyography revealed a right peroneal neuropathy at the knee with conduction block, mild right median neuropathy, and mild right ulnar neuropathy. The patient had hypogonadism with low total and free testosterone levels and hyperparathyroidism due to end-stage renal disease. Skeletal survey and positron emission tomography (PET) computed tomography (CT) were negative for osteosclerotic lesions. CT abdomen showed ascites, liver cirrhosis, splenomegaly, and atrophic kidneys. Pulmonary function tests revealed restrictive defect. He was started on cyclophosphamide 500 mg, bortezomib 2.3 mg, and dexamethasone 40 mg for treatment of multiple myeloma. Later cyclophosphamide was replaced with thalidomide 100 mg daily due to intolerance. The patient died 9 months after diagnosed with acute on chronic kidney disease due to end-stage renal failure and advanced multiple myeloma.

 > Discussion Top

POEMS syndrome is a multisystem disorder associated with plasma cell neoplasm. It affects both males and females (M>F) with age ranging from 50 to 60 years, and the majority of the cases are described in the Japanese population.[2] [Table 1] describes the major and minor criteria and other symptoms of POEMS syndrome.[1] Polyneuropathy is sensorimotor type and observed in all patients (100%).[1] Electromyography shows demyelination and axonal degeneration, but conduction block is rare which was seen in our patient.[1] Organomegaly is present in 45% of the patients and may present as enlarged liver, spleen, or lymph nodes.[1] Endocrinopathy was observed in 67–84% of the patients and the most frequent abnormality was hypogonadism.[1] Other endocrine abnormalities reported were adrenal insufficiency, hyperprolactinemia, hypothyroidism, and diabetes mellitus.[1]
Table 1: Criteria for the diagnosis of POEMS syndrome[1]

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The heavy chain of plasma cell dyscrasia in POEMS syndrome is IgA in 50% of the cases and light chain is always lambda.[2] In the study by Dispenzieri et al., 85% of the patients (N = 97) usually present with high serum M-protein concentrations and all the individuals had lambda (λ) light chains, and heavy chain was either IgA (44) or IgG (40) or IgM (1) type.[2] Similar to our case, Dursun et al. reported a case of POEMS syndrome with an IgG kappa type of monoclonal protein and the absence of typical skin and bone lesions.[3] In another two cases of POEMS syndrome presenting with IgG kappa gammopathy, one patient had diffuse cutaneous hyperpigmentation and thickening of skin without polyneuropathy and another patient had mixed osteosclerotic/lytic lesions and severe sensorimotor neuropathy.[4],[5]

Renal involvement in POEMS syndrome frequently presents as chronic renal insufficiency with mild proteinuria and/or microscopic hematuria or rarely as acute renal failure with severe anasarca.[6] Our patient had a history of chronic kidney disease, suddenly presented with rapidly progressive renal failure that advanced to end-stage renal disease requiring hemodialysis due to lack of response to chemotherapy. The typical renal lesion reported in POEMS syndrome patients includes membranoproliferative glomerulonephritis-like lesions, thrombotic microangiopathy, and mesangiolytic glomerulonephritis.[6] By immunofluorescent microscopy, no immunoglobulins or complement deposits are found.[6] Our patient had FSGS, an uncommon renal lesion in POEMS syndrome patients. To our knowledge, there is one case of POEMS syndrome with renal involvement reported to have FSGS, and the patient had hemodialysis due to poor response to steroids.[7] Higashi et al. reported a patient who had POEMS syndrome with normal VEGF levels.[8]

The major therapeutic approach of POEMS syndrome is the treatment of the plasma cell disorder including radiation therapy of plasmacytoma, steroids, and alkylating agents.[9] Sanada et al. reported that high-dose chemotherapy with melfalan followed by peripheral blood stem cell transplantation has been successful in some cases.[10] Bortezomib followed by melfalan and dexamethasone or autologous transplant was used for patients with POEMS syndrome at high risk, for example, those with renal failure, severe pulmonary hypertension, and refractory ascites.[11]

 > Conclusion Top

POEMS syndrome is an uncommon paraneoplastic syndrome, which may present with diverse clinical and pathological manifestations. Renal involvement in POEMS syndrome may be severe and aggressive requiring hemodialysis. Taking a detailed history and physical examination and a thorough diagnostic evaluation, including serum immunoelectrophoresis, serum cytokines and growth factors, skeletal survey, PET CT, and bone marrow biopsy can guide us in differentiating POEMS syndrome from other diseases.

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There are no conflicts of interest.

 > References Top

Dispenzieri A. POEMS syndrome: 2014 update on diagnosis, risk-stratification, and management. Am J Hematol 2014;89:214-23.  Back to cited text no. 1
Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR, et al. POEMS syndrome: Definitions and long-term outcome. Blood 2003;101:2496-506.  Back to cited text no. 2
Dursun B, Artac M, Varan HI, Akkaya BK, Karpuzoglu G, Suleymanlar G. An atypical case of POEMS syndrome with IgG kappa M protein and end stage renal failure. Int Urol Nephrol 2005;37:581-5.  Back to cited text no. 3
Morrow JS, Schaefer EJ, Huston DP, Rosen SW. POEMS syndrome: Studies in a patient with an IgG-kappa M protein but no polyneuropathy. Arch Intern Med 1982;142:1231-4.  Back to cited text no. 4
Romas E, Storey E, Ayers M, Byrne E. Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin change (POEMS) syndrome with IgG kappa paraproteinemia. Pathology 1992;24:217-20.  Back to cited text no. 5
Nakamoto Y, Imai H, Yasuda T, Wakui H, Miura AB. A spectrum of clinicopathological features of nephropathy associated with POEMS syndrome. Nephrol Dial Transplant 1999;14:2370-8.  Back to cited text no. 6
Heck D, Mergen M, Ganner A, Pelisek J, Mader I, Weiller C, et al. POEMS syndrome, calciphylaxis and focal segmental glomerulosclerosis-VEGF as a possible link. BMC Neurol 2014;14:210.  Back to cited text no. 7
Higashi AY, Nogaki F, Kato I, Ono T, Fukatsu A. Serial renal biopsy findings in a case of POEMS syndrome with recurrent acute renal failure. Clin Exp Nephrol 2012;16:173-9.  Back to cited text no. 8
Soubrier M, Sauron C, Souweine B, Larroche C, Wechsler B, Guillevin L, et al. Growth factors and proinflammatory cytokines in the renal involvement of POEMS syndrome. Am J Kidney Dis 1999;34:633-8.  Back to cited text no. 9
Sanada S, Ookawara S, Karube H, Shindo T, Goto T, Nakamichi T, et al. Marked recovery of severe renal lesions in POEMS syndrome with high-dose melphalan therapy supported by autologous blood stem cell transplantation. Am J Kidney Dis 2006;47:672-9.  Back to cited text no. 10
Li J, Zhang W, Kang WY, Cao XX, Duan MH, Zhou DB. Bortezomib and dexamethasone as first-line therapy for a patient with newly diagnosed polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes syndrome complicated by renal failure. Leuk Lymphoma 2012;53:2527-9.  Back to cited text no. 11


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