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Pearls & Oy-sters: POEMS Syndrome

The diagnostic challenge to early recognition in POEMS is the ubiquity of its initial presenting symptom, polyneuropathy, which leads to a broad differential. Screening for a plasma cell disorder is critical in the diagnostic work-up of any neuropathy. Pattern recognition can also aid in early diagnosis. POEMS is marked by a subacute, symmetric, often painful, sensorimotor neuropathy frequently with pronounced weakness... Although there are no randomized controlled trials, treatment for POEMS is based on treating the underlying plasma cell neoplasm with myeloma-adapted regimens.

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 Doctors Told Him He Was Going to Die. Then A.I. Saved His Life

For months, he had been battling a rare blood disorder called POEMS syndrome, which had left him with numb hands and feet, an enlarged heart and failing kidneys. Every few days, doctors needed to drain liters of fluid from his abdomen. He became too sick to receive a stem cell transplant — one of the only treatments that could have put him into remission.

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POEMS

To diagnose POEMS syndrome, the doctor will need to perform a thorough physical examination where they will look for: skin and hair changes; evidence of fluid build-up; enlargement of specific glands and organs such as lymph glands, liver and/or the spleen; signs of optic nerve swelling; signs of nerve damage. Blood and urine tests, imaging scans and biopsies may also be carried out to confirm the diagnosis.

POEMS Syndrome

A diagnosis of POEMS is determined by the presence of a monoclonal plasma cell disorder, peripheral neuropathy, and one or more of the following: osteosclerotic bone lesions, organomegaly, endocrinopathy, skin changes, increased levels of vascular endothelial growth factor, and swelling.

POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management

(POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease.

POEMS syndrome: 2021 Update on diagnosis, risk-stratification, and management

In summary, POEMS syndrome is an important paraneoplastic syndrome associated with a clonal plasma cell neoplasm. Making the diagnosis can be a challenge, but a good history and physical examination followed by appropriate testing—most notably radiographic assessment of bones, measurement of VEGF, and careful analysis of a bone marrow biopsy—can differentiate this syndrome from other conditions like CIDP, immunoglobulin light chain amyloidosis, and MGUS neuropathy. Once the diagnosis is made, attention to supportive care and treatments that are active in MM are essential; however, application of neurotoxic MM therapies should be used in the context of a clinical trial or at the time of relapsed or resistant disease.

POEMS Syndrome: A Case Report and Review of the Literature

Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein elevation, and skin changes (POEMS) syndrome is a rare multisystem disorder that occurs due to an underlying plasma cell dyscrasia. A diagnosis is made with the presence of two mandatory criteria and at least one major and one minor criterion.

POEMS syndrome: clinical update

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a rare paraneoplastic syndrome, caused by a plasma cell proliferative disorder, which is most commonly lambda restricted. The neurological hallmark, which forms one of the mandatory criteria for diagnosis, is a subacute onset demyelinating neuropathy, which can be rapidly disabling and painful.

POEMS Syndrome: Real World Experience in Diagnosis and Systemic Therapy

POEMS (Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) syndrome is a rare plasma cell disorder.1 It is actually a paraneoplastic syndrome caused by an underlying clonal plasma cell disorder which is difficult to recognize, underdiagnosed, and due to the rarity of the disease, outcome analyses are challenging and usually require multicenter cooperation. The association of the pro-inflammatory cytokine, vascular endothelial growth factor (VEGF) in POEMS is well documented.

Update on the Diagnosis and Treatment of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome

POEMS syndrome should be considered in the differential diagnosis for patients who have peripheral neuropathy and paraproteinemia among other multisystem manifestations. The syndrome can be debilitating if not recognized early in its course; thus, appropriate diagnosis and treatment are important for optimal clinical outcomes.

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Autoimmune Association

POEMS syndrome can be misdiagnosed because the signs and symptoms mimic those of other disorders. POEMS syndrome progresses rapidly and may become life-threatening, so early diagnosis is important.

Myeloma UK

What causes POEMS syndrome? The exact causes of POEMS syndrome are not well understood. Although all patients have abnormal plasma cells producing paraprotein, this is not enough to explain the many features of POEMS syndrome. Various cytokines (chemical messengers) are thought to play a role in causing damage to the different tissues and organs involved in the syndrome. This includes the cytokine VEGF that is found in higher levels in POEMS syndrome patients.