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CASE REPORT Table of Contents  
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POEMS syndrome: A rare entity


1 Department of Pathology, Guntur Medical College, Guntur, Andhra Pradesh, India
2 Department of Pathology, Guntur Medical College, Guntur; Department of Pathology, Siddartha Medical College, Vijayawada, Andhra Pradesh, India

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Date of Submission23-Aug-2021
Date of Decision04-Nov-2021
Date of Acceptance05-Nov-2021
Date of Web Publication13-Jan-2023
 

   Abstract 


POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. The diagnosis of POEMS syndrome can be a challenge. A good history, physical examination, and appropriate testing can aid in establishing its diagnosis. We are presenting the case of a 75-year-old man who was diagnosed with POEMS syndrome.

Keywords: Paraneoplastic syndrome, plasma cell neoplasm, POEMS syndrome, polyneuropathy


How to cite this URL:
Devi CP, Stephenson BR, Kumari P R, Vani PG, Madhooli SP. POEMS syndrome: A rare entity. Indian J Pathol Microbiol [Epub ahead of print] [cited 2023 Feb 5]. Available from: https://www.ijpmonline.org/preprintarticle.asp?id=367709





   Introduction Top


POEMS syndrome, also called Crow–Fukase syndrome, is a paraneoplastic syndrome associated with plasma cell neoplasm. The acronym POEMS stands for Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, Skin Changes; however, these components are not all required for the diagnosis. It is a rare disease estimated to account for <1% of plasma cell neoplasms.[1]


   Case History Top


A 75-year-old, diabetic man presented with anemia and came to clinical pathology with a request for bone marrow examination. Peripheral smear examination showed microcytic hypochromic anemia of a severe degree [Figure 1]. Bone marrow examination revealed 10 to 13% plasma cells [Figure 2]. Concurrently, the patient was also investigated for complaints of polyneuropathy with nerve conduction studies, which revealed bilateral symmetrical sensorimotor axonal polyneuropathy. A skeletal survey showed multiple osteosclerotic bone lesions were found [Figure 3]. On further investigations, his HbA1c and thyroid-stimulating hormone levels were raised. Serum protein electrophoresis showed a suspicious non-discrete protein band in the gamma globulin region (0.3 g/dL) [Figure 4]. Immunofixation electrophoresis identified a very faint non-discrete protein band in the gamma globulin region of IgA and kappa lane corresponding to a possible “M” spike [Figure 5]. Overall, the patient was confirmed to have polyneuropathy, monoclonal plasma cell proliferative disorder, osteosclerotic lesions, and endocrinopathy (diabetes and hypothyroidism). Hence, the diagnosis of POEMS syndrome was made adherent to the World Health Organization (WHO) 2017 criteria for POEMS syndrome [Table 1].
Figure 1: Peripheral smear showing microcytic hypochromic anemia

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Figure 2: Bone marrow aspiration shows 10 to 13% plasma cells (inset: binucleate plasma cells)

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Figure 3: Skeletal survey revealed multiple osteosclerotic bone lesions

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Figure 4: Protein Electrophoresis showed suspicious non-discrete band in gamma globulin region, possibly a monoclonal gammopathy

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Figure 5: Immunofixation Electrophoresis identifies a very faint non-discrete protein band in gamma globulin region of IgA and Kappa lane corresponding to ? 'M' spike

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Table 1: Criteria for the diagnosis of POEMS Syndrome

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   Discussion Top


The diagnosis of POEMS syndrome is based on a constellation of clinical and laboratory features and it will be missed if not considered. Most notably, the presence of neuropathy along with monoclonal proteins; thrombocytosis; anasarca; papilledema should warrant an in-depth search for POEMS syndrome. The recommended minimum testing to define a baseline and for future assessments was proposed by Dispenziari.[2]

Distinguishing POEMS syndrome from other differential diagnoses is necessary because the treatment and expected treatment-related morbidities are quite different. If a patient with POEMS syndrome is incorrectly deemed to have MGUS (monoclonal gammopathy of undetermined significance) or smoldering myeloma, the monoclonal plasma cells will not be targeted by the therapy. This will result in worsening of existing symptoms and accumulation of further elements of the paraneoplastic syndrome. In contrast, if a patient with POEMS syndrome is incorrectly labeled as plasma cell myeloma or plasmacytoma, the standard therapy for these disorders results in increased treatment-related morbidity.

Other differential diagnoses include chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), Waldenstrom's macroglobulinemia, immunoglobulin light chain amyloidosis, chronic ataxic neuropathy with ophthalmoplegia (CANOMAD), M-protein, cold agglutinins, and anti-disialosyl antibodies).[3]

The etiology and pathogenesis of POEMS syndrome are not well understood; however, markedly elevated levels of vascular endothelial growth factor (VEGF) appear to be an important pathogenic factor. Helpful cut-offs for plasma and serum VEGF levels to diagnose POEMS syndrome are 200 pg/mL (specificity 95%, sensitivity 68%).[4]

Wang et al.[5] have identified N-terminal propeptide of type I collagen (P1NP) as a novel marker for the diagnosis and follow-up of patients with POEMS. P1NP is the cleavage product of type 1 collagen, which constitutes 90% of the bone protein. It is a newly emergent marker of bone formation and has shown its clinical value in detecting osteosclerotic metastases. It has also proved to be a useful marker in standardizing the evaluation of osteosclerotic lesions in POEMS syndrome with a specificity of 91.5% and a sensitivity of 80%.

In most cases, POEMS syndrome is a chronic and progressive disease with a 5-year survival rate of 60 to 94%. Several clinical factors are associated with shorter survival, including extravascular fluid overload, clubbing, and respiratory symptoms.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition). IARC: Lyon; 2017. p. 256-7.  Back to cited text no. 1
    
2.
Dispenzieri A. POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management. Am J Hematol 2019;94:812-27.  Back to cited text no. 2
    
3.
Brown R, Ginsberg L. POEMS syndrome: Clinical update. J Neurol 2019;266:268-77.  Back to cited text no. 3
    
4.
D'Souza A, Hayman SR, Buadi F, Mauermann M, Lacy MQ, Gertz MA, et al. The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome. Blood 2011;118:4663-5.  Back to cited text no. 4
    
5.
Wang C, Zhou YL, Cai H, Cheng XQ, Zhang W, Kang WY, et al. Markedly elevated serum total N-terminal propeptide of type I collagen is a novel marker for the diagnosis and follow up of patients with POEMS syndrome. Haematologica 2014;99:e78-80.  Back to cited text no. 5
    

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Correspondence Address:
Chaganti P Devi,
Syamala Nagar 5th Line, Pattabhipuram, Guntur, Andhra Pradesh - 522 006
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_857_21



    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
 
 
    Tables

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