POEMS syndrome: How is it treated?

Are there any new developments in the treatment of POEMS syndrome?

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Answer

The most important development in the treatment of POEMS syndrome — a rare multisystem disorder — is the benefit from a combination of high-dose chemotherapy and stem cell transplant. This combination treatment may not be appropriate for all people with POEMS syndrome. But it shows great promise as a new therapeutic option for people with POEMS syndrome.

The term POEMS is an acronym for the disorder's most common symptoms:

• Polyneuropathy — numbness, tingling and weakness in the hands and feet
• Organ enlargement (organomegaly)
• Endocrinopathy — a disease affecting hormone-producing (endocrine) glands
• Monoclonal gammopathy — a collection of abnormal blood protein cells
• Skin changes — such as increased skin pigment, increased body hair, thickening of the skin

POEMS syndrome typically affects men older than age 50. It is so rare that it often goes undiagnosed or is misdiagnosed as multiple myeloma or chronic inflammatory demyelinating polyneuropathy (CIDP). The exact cause of POEMS syndrome isn't clear. But researchers believe it involves an overgrowth of bone marrow cells (plasma cells), which produce chemicals that damage other parts of the body.

Treatment for POEMS syndrome may include:

• Radiation therapy
• Chemotherapy
• Corticosteroids
• Hormone replacement therapy
• Physical therapy
• Bone marrow transplant

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