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Case Reports
. 2022 Nov 7;12(6):69-72.
doi: 10.55729/2000-9666.1121. eCollection 2022.

POEMS Syndrome - A Diagnostic Dilemma with Challenging Presentation

Mosunmoluwa Oyenuga  1 , Mohamed M G Mohamed  1 , Rushin Patel  1 , Sara Sartaj  1 , Sandeep Sen  2 , Alexandre Lacasse  1 , Vikas Kumar  3
Affiliations
Case Reports

POEMS Syndrome - A Diagnostic Dilemma with Challenging Presentation

Mosunmoluwa Oyenuga et al. J Community Hosp Intern Med Perspect. .

Abstract

Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes (POEMS) syndrome is a rare disorder with multiple presentations and a constellation of symptoms. We present a 62 year-old female who presented to the Emergency Department for acute dyspnea. Chest Xray showed sclerotic lesions in the ribs and thoracic spine. Further imaging studies with computed tomography (CT) and positron emission tomography (PET) scans were suggestive of a benign process. Improvement was seen with supportive management. A few months later, patient developed neurological symptoms with reduced exercise tolerance. Mixed demyelinating and axonal polyneuropathy was diagnosed by electromyography. Further work up with bone marrow biopsy and immunochemistry testing revealed lambda and kappa plasma cell disorder, with elevated vascular endothelial growth factor (VEGF). Patient was diagnosed with POEMS and initiated on chemotherapy. POEMS syndrome is commonly missed due to its rarity and varied clinical presentations. VEGF plays a crucial role in the diagnosis. Management requires a multidisciplinary approach.

Keywords: Demyelinating; Endocrinopathy; M-protein; Neuropathy; Organomegaly; POEMS syndrome; Plasma cell disorder; Polyneuropathy; Skin changes syndrome; VEGF; Vascular endothelial growth factor.

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Conflict of interest statement

Conflcits of interest None.

Figures

Fig. 1
Fig. 1
Positron emission tomography (PET) scan with increased metabolic activity to the right femur.
Fig. 2
Fig. 2
Nerve Conduction Study illustrating a) temporal dispersion of the left peroneal nerve b) conduction block in the right ulnar motor nerve.
Fig. 3
Fig. 3
Magnetic Resonance Imaging (MRI) brain showing diffuse thickening and enhancement of the dura.
See this image and copyright information in PMC

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