POEMS Syndrome

Overview

What is POEMS syndrome?

POEMS syndrome is a blood disorder that affects multiple systems in the body. In this rare condition, abnormal plasma cells cause elevated levels of antibody proteins to accumulate in the blood. Having too many of these proteins in the blood can damage nerves and cause several organ systems to malfunction.

POEMS syndrome is rare and can be life-threatening. It is also known as osteosclerotic myeloma, Crow-Fukase syndrome and Takatsuki syndrome.

Symptoms and Causes

What causes POEMS syndrome?

Abnormal growth of antibody making cells called plasma cells that live in your bones. No one knows what causes these plasma cells to grow abnormally.

What are the signs and symptoms of POEMS syndrome?

POEMS syndrome gets its name from the most common signs and symptoms experienced by people with the disorder:

  • Polyneuropathy: Nerve damage that primarily affects the arms and legs causing weakness (also called peripheral neuropathy)
  • Organomegaly: Enlargement of internal organs, including liver and spleen
  • Endocrinopathy: Abnormal levels of hormones produced by the endocrine glands
  • Monoclonal gammopathy: Abnormal growth of plasma cells in the bones. These cells produce a “monoclonal gammopathy” that can be detected in blood or urine tests
  • Skin changes: Growth of skin blood vessels particularly on the chest that look like small cherries

People with POEMS syndrome experience different symptoms depending on the parts of the body the disorder affects. These symptoms can include:

  • Fluid retention throughout the body
  • Numbness
  • Pain
  • Reproductive system problems and improperly functioning sex organs
  • Weakness
  • Vision problems
  • Feeling very tired
  • Loss of muscle

How common is POEMS syndrome?

POEMS syndrome is rare. It occurs in more men than women and typically develops in people in their 40s and 50s. Patients with a diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) should be tested for POEMS syndrome.

Diagnosis and Tests

How is POEMS syndrome diagnosed?

A doctor diagnoses POEMS syndrome with a medical history and several tests. These tests include:

  • Biopsy: A doctor takes a sample of bone marrow and looks at it for abnormal plasma cells.
  • Electromyelogram (EMG): This is a special test that measures nerve function.
  • Blood and/or urine tests: A doctor takes a sample of blood or urine to check the levels of proteins and other substances.
  • Imaging: Tests such as X-rays and CT scans enable doctors to look for bone lesions (abnormal thickening or hardening).
  • Physical exam: During a physical exam, a doctor looks for physical signs of POEMS syndrome, including swelling, skin changes, and organs that are larger than normal.
  • Other tests as necessary: These might include breathing tests, echocardiograms, or endocrine tests, depending on symptoms.

Management and Treatment

How is POEMS syndrome managed or treated?

Treatment for POEMS syndrome aims to manage symptoms. Therapy may include:

  • Chemotherapy: Anti-cancer drugs will slow or eliminate the abnormal plasma cells. Treatment is similar to the treatment of a cancer of the plasma cell called multiple myeloma. These treatments are extremely effective and generally well tolerated with few side effects. There are also drugs called immunotherapy that help your immune system kill off the bad plasma cells.
  • Radiation therapy: Doctors use high levels of radiation to kill the bad plasma cells.
  • Physical therapy: This treatment may help with mobility issues brought on by the neuropathy.
  • Other medications. Steroids and/or diuretics (to relieve swelling) may also be recommended.

What complications are associated with POEMS syndrome?

Complications of POEMS syndrome depend on the symptoms a person experiences. The treatments may cause complications of their own.

In some people with POEMS syndrome, nerve damage and weakness gets worse over time and can severely limit mobility. If too much fluid builds up near the lungs or chest cavity, it can cause chest pain and breathing trouble. Effective treatment will stop the nerve damage and help alleviate other symptoms of the disease as well. Nerve damage may slowly improve over time.

Prevention

What are the risk factors for POEMS syndrome?

There are no known risk factors for POEMS syndrome.

Can POEMS syndrome be prevented?

Because doctors do not know what causes the bad plasma cells that cause POEMS syndrome, you cannot prevent it.

Outlook / Prognosis

What is the prognosis (outlook) for people with POEMS syndrome?

The outlook for people with POEMS syndrome varies depending on the parts of the body affected and the degree to which they are affected. Effective treatment that will kill the bad plasma cells will prolong survival. Treatment can also ease symptoms to improve your quality of life.

Living With

When should I call the doctor regarding POEMS syndrome?

Contact your doctor if you experience the signs and symptoms of POEMS syndrome, especially weakness in the arms and legs.

What questions should I ask my doctor about POEMS syndrome?

If you have POEMS syndrome, you may want to ask your doctor:

  • What are my treatment options?
  • What should I do to minimize or manage side effects?
  • What support resources are available to me?
  • Should I see a specialist in plasma cell disorders to help guide my treatment

When can I go back to my regular activities if I have POEMS syndrome?

POEMS syndrome can affect your ability to maintain your usual activities. Your doctor will help you understand how the disorder will affect your daily life.

Resources

What resources are available for a person with POEMS syndrome?

You may find useful information at:

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Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy