POEMS syndrome: clinical update

Abstract

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. A number of multi-system features are also characteristic of this disorder, and certainly not restricted to those included in its acronym, which though limited, remains a useful and memorable name, helping distinguish POEMS syndrome from other paraproteinaemic neuropathies. The discovery of vascular endothelial growth factor (VEGF) in association with POEMS syndrome has been extremely useful in aiding clinical diagnosis, and monitoring response to treatment, as well as helping understand the underlying mechanism of disease. Interestingly, however, treatment targeting VEGF has been disappointing, suggesting other disease mechanisms or inflammatory processes are also important. Current understanding of the pathogenesis of POEMS syndrome is outlined in detail in the accompanying article by Cerri et al. Here, we review the clinical features of POEMS syndrome, differential diagnosis and available treatment options, based on current literature.

High-Resolution Nerve Ultrasound Abnormalities in POEMS Syndrome—A Comparative Study

“…Moreover, polyneuropathy presents often as the initial and isolated symptom in POEMS syndrome and is mostly symmetric, distal, painful and rapidly progressive. Since treatment is different from CIDP, an accurate diagnosis is important [ 6 ].…”

No surgeon is an island, no echo stands alone

“…Oncology shines brightly, shares holders greatly and teaches a very good example that demonstrates what happens if the code for the adequate translation of symptoms and signs gets lost. The vascular system and/or the inner and outer skins and surfaces of the human body may get involved in a cancer-mediated immune stress response, which orchestrates a socalled para-neoplastic manifestation [11][12][13]. Thus the underlying cancer (colon, lung, breast, prostate, kidney-cell cancer) provokes dermatitis, enteritis, thrombosis etc.…”

“…Thus the underlying cancer (colon, lung, breast, prostate, kidney-cell cancer) provokes dermatitis, enteritis, thrombosis etc. Therefore, it is essential for medical teaching and education to foster awareness of this type of cancer-induced phenomena [11][12][13]. Otherwise, the lack of code and inability to adequately read the symptoms and signs may have major consequences for patients.…”

“…5). Man involves him-or herself to bridge his or her existence and invents stories and myths about time, space, atoms, particles, waves, fields, forces, circuits, melodies, colours, structures, speeds, lights, systems, gods, religions, politics, sciences, arts, computers, cell phones, tablets and the poly-semiotic performance of oncology, women, men, children, nuts and shells ( [12][13][14][15][16]; Fig. 5).…”

Missed diagnosis of POEMS syndrome with onset of progressive fatigue and numbness: a case report

“…Other symptoms related to POEMS syndrome include papilledema, extravascular volume overload, sclerotic bone lesions, and thrombocytosis/erythrocytosis (PEST); and increasing concentration of vascular endothelial growth factor (VEGF); a predisposition toward thrombosis; and abnormal pulmonary function tests. 2 Consequently, patients are often misdiagnosed with diabetic peripheral neuropathy or hypothyroidism at their first visit to the endocrinology department. We herein describe a patient who presented with progressive fatigue and numbness in the lower extremities and was misdiagnosed with diabetes and diabetic peripheral neuropathy.…”