POEMS Syndrome

What is POEMS syndrome?

POEMS syndrome is a rare blood disorder that can impact multiple body systems. With POEMS, your body makes abnormal plasma cells that multiply out of control. These cells release a substance called a monoclonal protein (or M-protein) into your blood. Having too many abnormal plasma cells and too much M-protein can damage your nerves and organs.

POEMS syndrome gets its name from its most common signs and symptoms:

• Polyneuropathy involves nerve damage throughout your body. With POEMS, the damage may cause pain or other unpleasant sensations in your legs, arms, feet or hands.
• Organomegaly is abnormally large organs. With POEMS, your liver, spleen and lymph nodes may become enlarged.
• Endocrinopathy is a hormone problem. With POEMS, your endocrine glands may release an abnormal amount of hormones.
• Monoclonal protein (M-protein) is the protein that builds up in your blood with POEMS. M-proteins accumulate when a single plasma cell makes too many copies, or clones, of itself. Each plasma cell releases M-proteins into your blood so that eventually you have too many of them.
• Skin changes that occur with POEMS include darkening, thickening and other changes.

POEMS syndrome is also called osteosclerotic myeloma, Crow-Fukase syndrome, PEP syndrome and Takatsuki syndrome.

Who does POEMS syndrome affect?

POEMS syndrome is rare. Anywhere from 300 to 300,000 people have it. POEMS syndrome typically affects people in their 50s and 60s. Although it affects people regardless of sex, it’s more common in men and people assigned male at birth (AMAB).

What are the signs and symptoms of POEMS syndrome?

The most common signs of POEMS syndrome are polyneuropathy, organomegaly, endocrinopathy, having too many M-proteins and skin changes. Everyone with POEMS has polyneuropathy and too many M-proteins. Other signs and symptoms vary.

Polyneuropathy

Symptoms of polyneuropathy are the most common sign of POEMS syndrome. Often, people notice pain that’s most intense in their legs and feet, but their arms and hands may also be affected. Polyneuropathy may also feel like:

• Weakness.
• Tingling.
• Numbness.
• Prickling.
• Burning.
• A pins-and-needles sensation.

Organomegaly

Imaging procedures may show that you have an enlarged:

• Liver (hepatomegaly).
• Spleen (splenomegaly).
• Lymph nodes (lymphadenopathy).

Endocrinopathy

Low levels of sex hormones are the most common sign of endocrinopathy with POEMS. Sex hormones include testosterone and estrogen. Other signs include:

• Problems with regulating blood sugar levels (for example, diabetes).
• Thyroid problems (for example, hypothyroidism).
• Addison’s disease (adrenal insufficiency).

Men and people AMAB may have fertility problems and develop large breasts (gynecomastia).

Women and people assigned female at birth (AFAB) may have missed periods (amenorrhea) and develop larger breasts. Your body may make more prolactin, the hormone that causes milk to come in during pregnancy. As a result, milk may leak from your nipples even if you’re not pregnant.

M-Protein

High levels of M-protein are a sign of POEMS. Too much M-protein can cause tumors to form in your blood. Excessive M-protein can cause your bones to thicken or harden (osteosclerosis).

Skin changes

A variety of skin changes may take place, including:

• Darker skin.
• Thicker skin.
• White fingernails.
• Increased hair growth on your face and legs.
• Growth of blood vessels (particularly on your chest) that look like small cherries.

Other symptoms

Other symptoms may include:

• Swelling in your arms and legs.
• Unexplained weight loss.
• Bone and joint pain.
• Vision problems.
• Trouble breathing.
• Fatigue.
• Fever.

Some people with POEMS syndrome have a condition affecting their lymph nodes called Castleman Disease (CD). Your healthcare provider may suspect POEMS if you have the symptoms of CD.

What causes POEMS syndrome?

With POEMS, you experience a range of symptoms and effects because of what’s called a monoclonal plasma disorder. With POEMS, an abnormal plasma cell makes copies of itself — so many copies that the cells can damage tissue. These cells release M-protein into your bloodstream so there’s too much of it. The excess cells and excess M-protein can harm multiple body systems.

Still, scientists don’t know what causes plasma cells to grow abnormally in the first place.

Researchers have discovered that a protein called vascular endothelial growth factor (VEGF) may play a role. The majority of people with POEMS syndrome have high levels of VEGF. Research is ongoing to discover the relationship between VEGF and POEMS syndrome.

How is POEMS syndrome diagnosed?

Your provider will ask about your medical history and symptoms. They’ll perform a physical exam to check for signs of POEMS syndrome.

Your provider may perform several tests, including:

• Blood and urine tests: Your provider may test a blood or urine sample to see if you have high levels of M-protein or VEGF. They may perform a blood test to see if you have an abnormal amount of blood cells or abnormal-looking blood cells that may be signs of POEMS.
• Imaging: X-rays and CT scans allow your provider to see if your bones have hardened or thickened. These changes can occur if you have too much M-protein.
• Bone marrow biopsy: A bone marrow biopsy can reveal if you have abnormal-looking plasma cells or a high number of plasma cells.
• Electromyogram (EMG): An EMG is a test that measures nerve function. It can help your provider diagnose polyneuropathy.

Your healthcare provider may perform additional tests depending on your symptoms, including breathing tests, echocardiograms or endocrine tests.

If you’ve been diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP), your provider may run tests to see if you have POEMS syndrome.

How is POEMS syndrome managed or treated?

There isn’t a cure for POEMS syndrome, but treatment can help manage symptoms. Treatments may include:

• Radiation therapy: Radiation directs high radiation levels at the abnormal plasma cells, killing them.
• Chemotherapy: Anticancer drugs, like chemotherapy, destroy abnormal plasma cells or slow their growth. Chemo treatment for POEMS is similar to cancer treatments for multiple myeloma. These treatments are highly effective and often don’t cause serious side effects.
• Autologous stem cell transplant: This procedure replaces your abnormal plasma cells with healthy stem cells. Stem cells develop into mature cells, like plasma cells. During an autologous stem cell transplant, your provider removes healthy stem cells from your body. Then, they destroy the abnormal plasma cells with chemo or radiation. After treatment, your provider places the stem cells back into your body so that they can mature into healthy plasma cells.
• Physical therapy: This treatment may help you with mobility issues you may be experiencing because of polyneuropathy.

Your healthcare provider may also prescribe steroids and diuretics (to relieve swelling) to ease symptoms.

What complications are associated with POEMS syndrome?

With POEMS syndrome, nerve damage and weakness may worsen over time, making it harder to get around. If too much fluid builds up near your lungs or inside your chest cavity, you may experience chest pain and breathing trouble.

Effective treatment can stop or slow nerve damage and also help ease other symptoms. Nerve damage may slowly improve over time.

What are the risk factors for POEMS syndrome?

There are no known risk factors for POEMS syndrome.

Can POEMS syndrome be prevented?

POEMS isn’t preventable.

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

Your outlook depends on factors like which parts of your body are impacted, how early you receive your diagnosis and treatment and how you respond to treatment.

Getting treated for POEMS is essential. Untreated POEMS syndrome can be life-threatening. On the other hand, treatment that effectively kills abnormal plasma cells can help you live longer. Treatment can also relieve symptoms and improve your quality of life.

How long can you live with POEMS syndrome?

The median survival rate for most people with POEMS is 14 years. This means that about half of people live less than 14 years and half of people live longer. Your life expectancy depends on your unique disease experience and your treatment.

Ask your healthcare provider about your likely outcomes as someone living with POEMS syndrome.

When should I call the doctor regarding POEMS syndrome?

Contact your provider if you experience any symptoms associated with POEMS syndrome, especially weakness in your arms and legs.

What questions should I ask my doctor about POEMS syndrome?

Questions you may ask include:

• How serious is my condition?
• 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 return to my regular activities if I have POEMS syndrome?

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

Is POEMS syndrome an autoimmune disease?

POEMS is a paraneoplastic syndrome, not an autoimmune disease. A paraneoplastic syndrome involves a variety of symptoms that occur because of an underlying disorder. With POEMS, the underlying issue is that abnormal plasma cells release too many M-proteins into your bloodstream (monoclonal plasma cell disorder). The signs and symptoms of POEMS result from the abnormal plasma cells and M-proteins that can harm tissue in your body.

Is POEMS syndrome the same as multiple myeloma?

Multiple myeloma is cancer that affects plasma cells. POEMS syndrome is a collection of symptoms resulting from abnormal plasma cells and too much M-protein. The survival rate for POEMS syndrome is better than the life expectancy associated with multiple myeloma.

Still, chemotherapy treatments for POEMS and multiple myeloma are often similar. Some of the symptoms are similar, too.

A note from Cleveland Clinic

POEMS syndrome is a complex diagnosis. It may take a while for your healthcare provider to determine that your symptoms result from POEMS syndrome instead of a different condition. Once you receive a diagnosis, work closely with your care team to decide which treatments provide the most symptom relief. Ask them to connect you with organizations or support groups that help individuals and families navigate diagnoses like POEMS syndrome.