What Is POEMS Syndrome?

Medically Reviewed by Jennifer Robinson, MD on June 12, 2024
Written by Julie Marks
4 min read

POEMS syndrome and multiple myeloma are two rare blood conditions that affect the plasma cells in your body. Plasma cells are a type of white blood cell that make special antibodies to help you fight infections.

With POEMS syndrome and multiple myeloma, abnormal plasma cells create too many antibody proteins in the blood. The antibodies don't work correctly and can’t help protect you from infections like normal antibodies do. This can damage nerves and affect other organs.

Multiple myeloma is a type of blood cancer. It happens when faulty plasma cells build up in the bone marrow (the soft, spongy tissue found in the middle of bones) and crowd out healthy blood cells.

A rare type of multiple myeloma, known as osteosclerotic myeloma, has a strong link to POEMS syndrome. In fact, doctors sometimes refer to POEMS syndrome as osteosclerotic myeloma.

POEMS is an acronym for these common symptoms that describe the disorder:

  • Polyneuropathy: This is nerve damage that generally affects the arms and legs.
  • Organomegaly: It means there’s an enlargement of your internal organs, including the spleen and liver.
  • Endocrinopathy: The endocrine glands produce an abnormal level of hormones.
  • Monoclonal gammopathy: It’s an abnormal growth of plasma cells in the bones that can be spotted in blood or urine tests.
  • Skin changes: Red spots that look like small cherries grow on the skin.

Symptoms of POEMS depend on which parts of the body are affected.

Some common problems include:

  • Pain
  • Numbness
  • Fluid retention
  • Weakness
  • Tiredness
  • Reproductive system issues
  • Sexual problems
  • Vision issues
  • Loss of muscle
  • Skin changes
  • More hair on the face or legs

Osteosclerotic myeloma and POEMS syndrome almost always happen together. Only a few cases of osteosclerotic myeloma without symptoms of POEMS have ever been reported. That’s why the terms POEMS syndrome and osteosclerotic myeloma are sometimes used for each other, even though they’re technically different conditions.

Osteosclerotic myeloma is extremely rare and makes up less than 3% of all cases of multiple myeloma. People with this type of cancer have osteosclerosis. This is a condition characterized by abnormal density and hardening of bones. Doctors don’t know exactly what causes osteosclerotic myeloma.

Though POEMS has a definite connection to osteosclerotic myeloma, research shows the syndrome isn’t linked to classic multiple myeloma. Almost all people (97%) with classic multiple myeloma have lytic bone lesions. This means their bones are soft, thin, and break easily. The tiny number of people who have POEMS have the opposite: bones that are abnormally dense and hard.

While classic multiple myeloma usually strikes when a person is in their 60s or 70s, POEMS syndrome typically affects someone in midlife – the median age is 50.

Research has suggested that people with POEMS syndrome have a median survival that’s about four times longer than that of those with classic multiple myeloma. Median survival measures the time from when a group of people get a disease diagnosis or start treatment, and the point when half of that group is still alive.

POEMS syndrome is extremely rare. According to some studies, only several hundred cases have ever been reported, but the disorder may be underdiagnosed.

The syndrome occurs more often in men than in women. It typically affects people in their 40s and 50s.

Complications of POEMS syndrome might depend on the symptoms you have. For example, people with POEMS syndrome have nerve damage. For some people, this damage makes them weak and have a hard time moving. Or if too much fluid builds up near the lungs, they can have breathing issues.

Doctors may use one or several of the following tests to diagnose POEMS syndrome:

  • Physical exam: Your doctor will look for signs of POEMS syndrome, such as skin changes or swelling.
  • Biopsy: Doctors remove a sample of your bone marrow and examine it for abnormal plasma cells.
  • Electromyogram (EMG): An EMG analyzes how well your nerves work.
  • Urine or blood tests: These tests can reveal abnormal levels of certain proteins.
  • Imaging: X-rays and CT scans help doctors spot bone lesions.
  • Other tests: Your doctor may recommend endocrine tests, breathing tests, or echocardiograms.

Treatments for POEMS syndrome keep symptoms at bay, but they won’t cure the condition. Some treatments doctors might recommend include:

  • Chemotherapy: It uses drugs to kill bad plasma cells.
  • Immunotherapy: This treatment uses your body’s immune system to target abnormal plasma cells.
  • Radiation therapy: High doses of radiation destroy the problem plasma cells.
  • Other medicines: Steroids or diuretics may be given to relieve swelling or other symptoms.
  • Physical therapy (PT): PT is sometimes used to help with mobility problems or weakness issues that are caused by neuropathy.
  • Stem cell transplant: With this procedure, high doses of chemo are given followed by an infusion of healthy blood stem cells from your own body.

The outlook for POEMS syndrome varies from person to person. Yours might depend on how severe your disease is and what body parts are affected. Treatments to kill faulty plasma cells can help you live longer and improve your quality of life.

The median survival for people with POEMS syndrome is more than 14 years. The outlook has improved a lot over the past 10 years and is likely to get even better with future medical advances.