POEMS Syndrome

What is POEMS syndrome?

POEMS syndrome is a rare blood disorder. It involves having too many abnormal plasma cells. Plasma cells are a type of white blood cell found in the bone marrow that produce antibodies to help fight infection.

POEMS syndrome can be challenging to diagnose because its symptoms are similar to many other conditions. Without treatment, POEMS syndrome can progressively damage your nerves and multiple organs. The condition is also referred to as osteosclerotic myeloma, PEP syndrome, Crow-Fukase syndrome and Takatsuki syndrome.

POEMS syndrome gets its name from the signs and symptoms that it commonly causes:

• Polyneuropathy: Nerve damage that affects peripheral nerves.
• Organomegaly: When some of your organs are larger than they should be.
• Endocrinopathy: When your endocrine system doesn’t release normal amounts of hormones.
• Monoclonal protein (M-protein): Refers to high M-protein levels in your blood.
• Skin changes.

Is POEMS syndrome a blood disorder?

POEMS syndrome is rare blood disorder. It is classified as a multisystem disorder because it can affect many organs and body systems, not just the nerves. Therefore, care often involves specialists in hematology, neurology and endocrinology, as well as nephrology, cardiology, physical therapy and other specialties, depending on your symptoms.

Multidisciplinary care of POEMS syndrome, where these specialists work together, is important as it allows every aspect of the condition to be considered within a single, coordinated treatment plan, rather than patients needing to navigate multiple separate clinics.

What are the symptoms of POEMS syndrome?

POEMS syndrome symptoms may include:

• Polyneuropathy: Symptoms may include numbness, tingling, shooting pain, burning, or prickling in your toes and feet. Your hands may become weak, making it difficult to hold on to things. As the condition worsens, standing or breathing may become difficult.
• Organomegaly: This is when your spleen, liver or lymph nodes become enlarged. Symptoms might include fatigue, pain, swelling and fullness.
• Endocrinopathy: Symptoms may include swelling in your arms and legs, a reduced sex drive, or discharge from your nipples.
• Monoclonal protein (M-protein): Abnormal plasma cells that produce M protein can damage or weaken your bones and other tissue.
• Skin changes: Changes may include darkening of your skin, red spots on your chest, nails turning white or more hair appearing on your face or legs.

What causes POEMS syndrome?

The exact cause of POEMS syndrome isn’t fully understood, but there may be a link between abnormal plasma cells and vascular endothelial growth factor (VEGF), a protein which helps new blood vessels to grow, because most people with POEMS syndrome also have high levels of VEGF.

Abnormal plasma cells may damage tissues, and they also release M-proteins into the blood. Together, these changes can damage different parts of the body.

High levels of VEGF can also affect blood vessels, causing them to leak into nearby tissue. This excess fluid may cause swelling of the organs and damage to your nerves.

POEMS syndrome can also cause serious health problems, such as:

• Pulmonary hypertension
• Restrictive lung disease
• Capillary leak syndrome

How is POEMS syndrome diagnosed?

POEMS syndrome can be difficult to diagnose and there are many other conditions that can cause similar symptoms.

As a result, diagnosis is often delayed. Many people undergo numerous tests, often over a long period of time, before the condition is accurately diagnosed.

POEMS syndrome is frequently diagnosed when the disease has progressed significantly, with severe nerve damage affecting mobility, highlighting the importance of early diagnosis and prompt specialist assessment.

To diagnose POEMS syndrome, your doctor will discuss your medical history, current medical concerns and symptoms in detail.

Tests to diagnose POEMS syndrome may include:

• Blood and urine tests: These can measure M-protein or VEGF levels and look for evidence of a plasma cell disorder.
• Imaging tests: CT scans or X-rays may show damage that M-proteins may have caused.
• Bone marrow biopsy: To detect abnormal plasma cells in the bone marrow.
• Electromyogram (EMG): To assess nerve and muscle function.
• Breathing tests: To see whether the lungs or respiratory muscles have been affected.
• Echocardiogram: To assess the structure and function of the heart.
• Endocrine tests: To assess the function of several endocrine glands.

Other tests may be given, depending on the symptoms you are experiencing.

How is POEMS treated?

Treatment focuses on managing the symptoms of POEMS syndrome and may include:

• Chemotherapy: This can destroy abnormal plasma cells or slow their growth.
• Immunotherapy: This treatment uses your body’s own immune system to destroy abnormal plasma cells.
• Radiation therapy: This may be used to treat bone lesions caused by abnormal plasma cells.
• Autologous stem cell transplant: This replaces abnormal plasma cells with healthy stem cells.
• Physical therapy: Movement can be restricted due to polyneuropathy, so physical therapy may help.
• Mental health support: Therapists can help you to manage the stress and anxiety that may come with living with a complex disease.

If POEMS syndrome is diagnosed promptly and appropriate treatment is given, many people experience significant improvement in their symptoms and have good long-term outcomes.

When should I talk to a doctor?

If you have a tingling or pins-and-needles feeling in your feet or legs that doesn’t go away, talk to your doctor. If you have been diagnosed with POEMS syndrome and your symptoms get worse, tell your doctor.