Treatment for POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes)

POEMS syndrome is a rare disorder of the blood which can cause a polyneuropathy (nerve symptoms such as numbness, tingling, pain, and muscle weakness) but can also involve many of the organs of the body, causing enlarged organs or organomegaly (usually liver, spleen, and lymph nodes), changes in hormone production or endocrinopathy (gynecomastia in men), abnormal blood protein (M-protein), and skin changes such as increased pigmentation or skin thickening. Its cause is not known. The quality of life of people with POEMS deteriorates because of progressive neuropathy, and accumulation of fluid in the limbs or in the abdominal cavity or cavity around the lungs. It is a potentially fatal disease, and serious complications can arise due to multiorgan failure. There is no established treatment regimen, but potentially effective treatments that have been tried include chemotherapy, irradiation, corticosteroids, thalidomide or lenalidomide, and blood stem cell transplantation. This review found no randomized controlled trials of treatments for POEMS syndrome. Prospective treatment trials are needed to establish the relative values of different treatments.

Authors' conclusions: 

There are no randomized or quasi-randomized controlled clinical trials of treatment for POEMS syndrome on which to base practice.

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Background: 

POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome is a rare cause of demyelinating and axonal mixed neuropathy with monoclonal plasma cell proliferative disorder and multiorgan involvement. The pathogenesis of POEMS syndrome is not well understood, but overproduction of vascular endothelial growth factor (VEGF), probably secreted by plasmacytomas, is likely to be responsible for most of the characteristic symptoms. POEMS syndrome is a potentially fatal disease, and patients' quality of life deteriorates because of progressive neuropathy, massive pleural effusion or ascites, or thromboembolic events. There is a need for efficacious therapy to improve prognosis. This is the first update of a review first published in 2008.

Objectives: 

To assess the effects of treatment for POEMS syndrome.

Search strategy: 

We searched the Cochrane Neuromuscular Disease Group Specialized Register (23 February 2012), CENTRAL (2012, Issue 2), MEDLINE (January 1966 to February 2012), EMBASE (January 1980 to February 2012) and CINAHL Plus (January 1937 to February 2012) for all papers on POEMS syndrome

Selection criteria: 

We sought all randomized and quasi-randomized controlled trials, and non-randomized controlled studies. Since we discovered no such clinical trials, we assessed and summarized all retrospective case series including five or more patients in the 'Discussion' section.

Data collection and analysis: 

Two review authors independently reviewed and extracted details of all potentially relevant trials with any treatment for POEMS syndrome. We then collated and summarized information on the outcome.

Main results: 

We found no randomized or non-randomized prospective controlled trials of treatment for POEMS syndrome. We summarized the results of retrospective case series containing five or more patients in the 'Discussion' section.

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