Definition

What is POEMS syndrome?

POEMS syndrome is a rare, mulitisystem disorder. POEMS is an acronym that stands for (P)olyneuropathy, disease affecting many nerves; (O)rganomegaly, abnormal enlargement of an organ; (E)ndocrinopathy, disease affecting certain hormone-producing glands that help to regulate sexual function, and certain metabolic functions; (M)onoclonal gammopathy or M proteins; and (S)kin abnormalities.

How common is POEMS syndrome?

POEMS syndrome affects men more often than women and usually occurs during the forties or fifties, although it has been reported in individuals in their twenties. The disorder was originally thought to be more common in Japan than in the United States and Europe. POEMS syndrome often goes unrecognized, making it difficult to determine the true frequency in the general population. POEMS syndrome is rare. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of POEMS syndrome?

The common symptoms of POEMS syndrome are:

  • Numbness, tingling and weakness in your legs — and over time, maybe in your hands — and difficulty breathing.
  • Enlarged spleen, liver or lymph nodes.
  • Abnormal hormone levels that can result in underactive thyroid (hypothyroidism), diabetes, sexual problems, fatigue, swelling in your limbs, and problems with metabolism and other essential functions.
  • Monoclonal plasmaproliferative disorder. Abnormal bone marrow cells (plasma cells) that produce a protein (monoclonal protein) that can be found in the bloodstream.
  • Skin changes. More color than normal on your skin, possibly thicker skin and increased facial or leg hair.

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes POEMS syndrome?

The cause of POEMS syndrome is not well understood. POEMS syndrome is a paraneoplastic disorder, which means that the signs and symptoms of the syndrome appear as a manifestation of the plasma cell disorder that the patient has (most patients are seen with osteosclerotic myeloma or monoclonal gammopathy of unknown significance); however, the mechanism by which this occurs is unknown. It is associated with a chronic overproduction of some substances known as pro-inflammatory cytokines (small molecules that act as messengers between cells to promote inflammation). These include interleukins (IL-1b, IL6) which are cytokines that help regulate immune responses, TNFα (Tumor Necrosis Factor alfa is a cytokine which is involved in the inflammatory process) and vascular endothelial growth factor (VEGF – a powerful vasodilator that may cause leaky blood vessels) which seem to be important in this disorder.

Risk factors

What increases my risk for POEMS syndrome?

Please discuss with your doctor for further information.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is POEMS syndrome diagnosed?

People who are suspected of having POEMS syndrome should first have their medical history evaluated and undergo a complete physical exam. Features suggestive of POEMS syndrome in the history include complaints of neurological symptoms, skin changes, signs of extravascular volume overload, and symptoms suggestive of an endocrine disorder (such as gynecomastia or irregular periods). A detailed physical exam should include an eye exam for papilledema; a neurologic exam; evaluation for enlargement of organs (organomegaly); examination of the skin; and evaluation for the presence of peripheral edema (swelling due to fluid accumulation), pleural or pericardial effusion (fluid build-up around the lungs or heart), ascites, clubbing (enlargement of the fingertips), heart failure, and cardiomyopathy.

A diagnosis of POEMS syndrome requires both the presence of polyneuropathy and a monoclonal plasma cell proliferative disorder, plus the presence of at least one major and one minor criterion on a physical exam, imaging, or laboratory evaluation.

Major criteria include:

  • An osteosclerotic or mixed sclerotic/lytic lesion seen on plain films or CT scan measuring at least 0.8 cm in the longest dimension
  • Castleman’s disease
  • Elevated serum or plasma vascular endothelial growth factor (VEGF) levels at least 3 to 4 times the upper limit of normal

Minor criteria include:

  • Organomegaly (enlargement of the spleen, liver, or lymph nodes)
  • Extravascular volume overload (peripheral edema, ascites, or pleural effusion)
  • Endocrinopathy (excluding diabetes mellitus or hypothyroidism)
  • Skin changes
  • Papilledema
  • Thrombocytosis or polycythemia

Many affected people are initially misdiagnosed as having other disorders, such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). In addition, a number of conditions are associated with a plasma cell disorder and polyneuropathy (with or without osteosclerotic bone lesions), and need to be distinguished from POEMS syndrome.

How is POEMS syndrome treated?

The treatment of POEMS syndrome is two pronged. The first is directed at treating the underlying plasma cell disorder (e.g. the osteosclerotic myeloma). The second is directed toward ameliorating the specific symptoms that are apparent in each individual. Treatment may require the coordinated efforts of a team of specialists.

The use of ionizing radiation (radiotherapy) or surgical removal (excision) or of osteosclerotic lesions that are localized (i.e., not widespread throughout the body) may temporarily (and sometimes permanently) lead to a remission of symptoms associated with POEMS syndrome.

In many cases, including those with widespread osteosclerotic lesions or diffuse bone marrow involvement, therapy with certain anticancer drugs (chemotherapy), like corticosteroids with cyclophosphamide or melphalan may alleviate symptoms associated with POEMS syndrome. Many patients may be offered high-dose chemotherapy with peripheral blood stem cell transplant.

Other treatment is symptomatic and supportive.

Investigational Therapies

Because POEMS syndrome is a plasma cell disorder (i.e. due to an overgrowth of plasma cells), it is a cousin disease to multiple myeloma. In the 2000s, there have been several new drugs to treat myeloma. These may be tried only with extreme caution in patients with POEMS syndrome, because the major side effect of several of these new drugs is peripheral neuropathy, the dominant symptom in patients with POEMS syndrome.

Additional therapies that have been used to treat POEMS syndrome include lenalidomide, thalidomide and bortezomib. The role of the following therapies is much less certain: anti-VEGF antibodies, interferon, intravenous immunoglobulin, plasmapheresis, and all-trans-retinoic acid.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage POEMS syndrome?

Please discuss with your doctor for further information.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Review Date: November 29, 2017 | Last Modified: November 29, 2017

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