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.

Name Description

  • 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.

Causes of 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.

Symptoms of POEMS Syndrome


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.


Imaging procedures may show that you have an enlarged:

  • Liver (hepatomegaly).

  • Spleen (splenomegaly).

  • Lymph nodes (lymphadenopathy).


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.


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.

Diagnosis of POEMS Syndrome

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.


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.

Treatment of POEMS Syndrome

Radiation therapy

Radiation directs high radiation levels at the abnormal plasma cells, killing them.


  • 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.

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