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.
Highlights
IntroductionThe diagnosis of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes), based on the current Dispenzieri diagnostic criteria [1], requires the presence of both mandatory criteria (a polyneuropathy and a monoclonal plasma cell-proliferative disorder, almost always lambda restricted), and at least one major and one minor criterion (Table 1)
The diagnosis of POEMS syndrome, based on the current Dispenzieri diagnostic criteria [1], requires the presence of both mandatory criteria, and at least one major and one minor criterion (Table 1)
POEMS syndrome has a median age of onset in the sixth decade and a slight male preponderance [2]
Summary
The diagnosis of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes), based on the current Dispenzieri diagnostic criteria [1], requires the presence of both mandatory criteria (a polyneuropathy and a monoclonal plasma cell-proliferative disorder, almost always lambda restricted), and at least one major and one minor criterion (Table 1). A number of small studies and case reports have shown mixed results with bevacizumab, including treatment failure (except VEGF response), and worsening of disease leading to death [67,68,69,70]. A small number of studies have suggested that short-term use of bevacizumab may be helpful as a bridge to definitive treatment such as ASCT, either alone or with other treatment such as thalidomide [72, 73] At this stage, the role of bevacizumab is unclear, and better understanding of the pathophysiology of POEMS syndrome is required before it can be recommended. Post-treatment, prognosis in POEMS syndrome is good; in a study of 291 patients treated at the Mayo clinic between 1974 and 2014, 10-year overall survival was 62%, with younger age, albumin > 3.2 g/dL and complete haematological response to treatment all associated with better outcomes [74]. Given the risk of relapse, close follow-up of clinical, radiological and biochemical parameters, is essential
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