POEMS Syndrome

POEMS syndrome is a rare blood disorder that damages your nerves and organs. The condition gets its name from its most common signs and symptoms:

• Polyneuropathy, nerve damage that affects peripheral nerves throughout your body
• Organomegaly, which is when some of your organs are larger than usual
• Endocrinopathy, when your endocrine system doesn’t release normal amounts of hormones
• Monoclonal protein or M-protein, which refers to high M-protein levels in your blood
• Skin changes

POEMS syndrome is a challenging condition. Many other health conditions may cause similar symptoms. People with this condition often go through rounds of tests over several months before healthcare providers can pinpoint POEMS syndrome.

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

Symptoms of POEMS syndrome

POEMS syndrome symptoms include:

• Polyneuropathy: Symptoms are numbness or sensations like sharp shooting pain, burning, tingling or prickling in your toes and feet. Your hands weaken so you can’t hold on to things. Later, the condition can make it hard to stand up or breathe.
• Organomegaly: Your liver, lymph nodes or spleen get larger. Symptoms may be fatigue, fullness, pain or swelling in your belly from an enlarged liver or spleen.
• Endocrinopathy: Symptoms include reduced sex drive, milky discharge from your nipples and swelling in your arms and legs.
• Monoclonal protein (M-protein): A buildup of this protein can weaken or damage your bones.
• Skin changes: Your skin may appear darker than usual. You may develop thick skin, red spots on your chest or have more hair on your face or legs. Your nails may turn white.

POEMS syndrome causes

Experts don’t know the exact cause. But they’re investigating a link between abnormal plasma cells and a protein that helps your body grow new blood vessels. They’re focused on this because most people with POEMS syndrome have high levels of abnormal plasma cells and vascular endothelial growth factor (VEGF).

Abnormal plasma cells can damage tissues. The cells also release M-proteins into your blood. Combined, abnormal plasma cells and M-proteins can damage different areas of your body. High VEGF levels can affect your blood vessels, so they leak fluid into nearby tissues. The excess fluid in tissues can cause swelling, make your organs get larger and damage your nerves.

Complications

POEMS syndrome may quickly get worse, increasing your risk of life-threatening issues, like:

• Capillary leak syndrome that can cause organ failure
• Restrictive lung disease
• Pulmonary hypertension

How doctors diagnose POEMS syndrome

A healthcare provider will ask about your symptoms and any current or past medical issues. They’ll do a physical exam and tests to check for signs of POEMS syndrome. Tests may include:

• Blood and urine tests: They’ll check your M-protein or VEGF levels. They may check for abnormal plasma cells.
• Imaging tests: X-rays and CT scans may show damage that M-proteins can cause.
• Bone marrow biopsy: This test may detect abnormal plasma cells in your bone marrow.
• Electromyogram (EMG): This test measures your nerve function.

You may have other tests, depending on your symptoms. These include breathing tests, echocardiograms and endocrine tests.

How is POEMS syndrome treated?

Treatment focuses on managing your symptoms and may include:

• Chemotherapy: This treatment destroys abnormal plasma cells or slows their growth.
• Immunotherapy: These medications use your own immune system to kill abnormal plasma cells.
• Radiation therapy: Radiation kills the abnormal plasma cells.
• Autologous stem cell transplant: This procedure replaces your abnormal plasma cells with healthy stem cells.
• Physical therapy: Polyneuropathy can make it hard for you to move around. Physical therapy may help.
• Mental health support: It can be stressful living with a rare disease with no immediate cure. You may feel anxious and depressed. Counselors may suggest ways you can manage your stress, anxiety and depression.

When should I seek care?

Talk to a healthcare provider if you have a tingling or pins-and-needles sensation in your feet or legs that doesn’t go away. If you have POEMS syndrome, tell your provider if your symptoms get worse.

What is the life expectancy for POEMS syndrome?

With prompt diagnosis and appropriate treatment, many people can live 10 or more years after diagnosis. Newer targeted and immune therapies are now available to treat POEMS and show additional promise. But you may have a different outcome, depending on your diagnosis and treatment. Your healthcare provider is your best source for understanding how long you may live with POEMS syndrome.