Abstract Details

Title Outcomes in POEMS patients: A case series

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P1 - Poster Session 1 (9:00 AM-5:00 PM)

Poster/Presentation
Number 401

Objective We aimed to determine variables associated with neurologic outcome in POEMS patients.

Background POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a rare, multi-system, paraneoplastic syndrome caused by plasma cell neoplasia. Polyneuropathy is mandatory for diagnosis and is often a presenting symptom, although it is often misdiagnosed as CIDP due to the presence of demyelinating features on electrodiagnostic (EDX) testing.

Design/Methods We conducted a retrospective case series of eight cases of POEMS syndrome diagnosed at our institution over a nine-year period. We collected data regarding clinical profiles, duration of disease prior to oncologic therapy, treatment, and outcomes, with the goal of identifying variables associated with favorable prognosis.

Results Patients ranged in age from 17 to 62 at presentation. The duration of disease prior to initiation of POEMS-specific treatment ranged from 7 to 27 months. Seven patients had received first-line therapy for CIDP with limited to no response. Bone imaging revealed lytic lesions in all patients. VEGF levels ranged from normal to elevated. The presence of organomegaly, endocrinopathy, and skin changes varied considerably. Electrodiagnostic studies, initially characterized by demyelinating features, showed secondary axonal changes in several patients at the time of POEMS diagnosis. Nerve biopsy was performed in one patient, which showed severe neuropathy with primary demyelination.

Conclusions We aimed to analyze if time to diagnosis, number of bone lesions, serum VEGF levels, EMG characteristics were associated with poor outcomes. We did not find any significant associations with these factors and poor neurologic outcomes (mRS). Our study was limited as it was a small case series. However, we emphasize the importance of considering the diagnosis of POEMS as a treatable, sub-acute neuropathy. The treatment of POEMS is targeted and specific and therefore awareness and consideration of this diagnosis is paramount.

Authors/Disclosures
Amna Ramzan, MD, MBBS PRESENTER	Dr. Ramzan has nothing to disclose.
Doyle Yuan, MD (Dallas VA Medical Center)	Dr. Yuan has nothing to disclose.
Kristin A. Brown, MD (University of Texas, Houston)	Dr. Brown has nothing to disclose.
Suur Biliciler, MD	Dr. Biliciler has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Efficient CME. Dr. Biliciler has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Argenyx. Dr. Biliciler has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for UCB.
Kazim A. Sheikh, MD (The University of Texas Health Science Center at Houston)	The institution of Dr. Sheikh has received research support from NIH. The institution of Dr. Sheikh has received research support from DoD.
Thy Nguyen, MD (University of Texas Health Science Center)	Dr. Nguyen has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for ArgenX. Dr. Nguyen has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for UCB. Dr. Nguyen has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion. Dr. Nguyen has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Takeda. Dr. Nguyen has received publishing royalties from a publication relating to health care.

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