Abstract Details

Title Not Your Average CIDP: When the Liver Writes POEMS

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P1 - Poster Session 1 (8:00 AM-9:00 AM)

Poster/Presentation
Number 9-014

Objective NA

Background Progressive polyradiculoneuropathy that mimics CIDP may result from a paraneoplastic etiology in a constellation of symptoms known as POEMS syndrome. It should be considered when findings are atypical, and standard treatments for CIDP are unsuccessful. A high index of suspicion is required from neurologists to recognize the syndrome in its sea of medical problems. POEMS syndrome results from a plasma cell dyscrasia, commonly a plasmacytoma involving the bone marrow. Extramedullary plasmacytomas account for less than 4% of cases, most frequently in the upper aerodigestive tract. Isolated hepatic plasmacytomas are exceedingly rare and have not previously been reported in association with POEMS syndrome. Treatment involves targeting the plasma cell dyscrasia with surgery, radiation therapy, or chemotherapy.

Design/Methods

A 55-year-old man presented after four months of progressive ascending length-dependent sensorimotor weakness with 0/5 lower extremity strength and 3/5 upper extremity strength without response to steroids, IVIg, or plasmapheresis. EMG/NCS showed a primarily axonal length-dependent sensorimotor polyneuropathy. Investigation showed CSF albuminocytologic dissociation, polycythemia, hypogonadism, a monoclonal IgG lambda gammopathy, splenomegaly, anasarca, and elevated VEGF. Biopsy of a 3cm hepatic lesion was consistent with a plasmacytoma.

Results

The patient met both mandatory criteria of polyneuropathy and plasma cell dyscrasia, major criteria of elevated VEGF and minor criteria including organomegaly, extravascular volume overload, endocrinopathy, polycythemia, and other classic symptoms including weight loss, venous thromboembolism. He was treated with chemotherapy and radiation therapy.

Conclusions Chronic progressive polyneuropathies that mimic CIDP and occur alongside plasma cell dyscrasias should raise suspicion for POEMS syndrome. The purpose of this case presentation is to report a unique extramedullary site of plasmacytoma in the liver, as our review of the literature shows only one other case of a hepatic plasmacytoma associated with polyneuropathy. This is the first reported case of an isolated hepatic plasmacytoma associated with POEMS syndrome.

Authors/Disclosures
William Remley, Jr., DO PRESENTER	Dr. Remley has nothing to disclose.
Xiaojun Li, MD (Tsinghua University)	Dr. Li has nothing to disclose.
Bhavin A. Patel, DO (UFHealth)	Dr. Patel has nothing to disclose.
Kyle W. Ruffing, MD, FAAN (University of Florida Department of Neurology)	Dr. Ruffing has nothing to disclose.

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