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

Kuwabara S, Dispenzieri A, Arimura K, Misawa S

Published Online:

October 8, 2008

POEMS syndrome is a rare disorder of the blood which can cause a polyneuropathy (nerve symptoms such as numbness, tingling, weakness, and pain) 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, 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 treatments tried include chemotherapy, irradiation, intravenous immunoglobulin, plasma exchange, corticosteroids, 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.

Abstract (click to read)

Background:

POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome is a rare cause of demyelinating and axonal mixed neuropathy with multiorgan involvement and monoclonal plasma cell-proliferative disorder. 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.

Objectives:

To provide the best available evidence from randomised controlled trials on treatment for POEMS syndrome.

Search strategy:

We searched the Cochrane Neuromuscular Disease Group Trials Register (March 2008), MEDLINE (from January 1966 to March 2008), EMBASE (from January 1980 to March 2008) and CINAHL (from January 1982 to March 2008) for randomized controlled trials, quasi-randomized trials, historically controlled studies, and trials with concurrent controls. We adapted this strategy to search MEDLINE from 1966 and EMBASE from 1980 for comparative cohort studies, case-control studies and trials, and case series.

Selection criteria:

All randomized and quasi-randomized controlled trials, and non-randomized controlled studies were sought. 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 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.

Authors' conclusions:

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

This record should be cited as:

Kuwabara S, Dispenzieri A, Arimura K, Misawa S. Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome. Cochrane Database of Systematic Reviews 2008, Issue 4. Art. No.: CD006828. DOI: 10.1002/14651858.CD006828.pub2

Assessed as up to date:

March 31, 2008

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Primary Review Group:

Neuromuscular Disease Group

Health topics:

Neurology > Peripheral neuropathy > Paraproteinaemic neuropathy

Cochrane Summaries^beta

Independent high-quality evidence for health care decision making

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

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