Everything You Need to Know About POEMS Syndrome

A Rare Multisystem Disease

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin abnormalities (POEMS) syndrome is a rare multisystem disease that causes a blood cell disorder and nerve damage, with several other effects.

Symptoms can vary, potentially involving abdominal swelling, shortness of breath, and pain. This condition develops during adulthood, at an average age of 40 to 60 years. The underlying cause is unknown.

Because it’s rare and causes a wide range of symptoms, POEMS syndrome isn’t usually recognized or diagnosed until several signs and symptoms develop. There is no cure for POEMS syndrome, but treatments can help alleviate some of the effects. This article explains what you can expect with POEMS syndrome. 

Person with hands on swollen abdomen

Mladen Zivkovic / Getty Images

What Is POEMS Syndrome?

Several organs and areas of the body are impacted by POEMS syndrome. The different effects are not directly related to each other, so symptoms of POEMS syndrome are wide-ranging. 

POEMS syndrome is named for its characteristic signs and symptoms: polyneuropathy, organomegaly, endocrinopathy/edema, monoclonal plasma cells and proteins, and skin changes.

Mandatory criteria (must be present for diagnosis) are:

  • Polyneuropathy: Damage of multiple nerves throughout the body can cause pain, loss of sensation, and muscle weakness. Polyneuropathy can also cause shortness of breath, problems chewing and swallowing, abdominal discomfort, constipation, or incontinence. 
  • Monoclonal plasma cells: Plasma cells normally make antibodies (immune proteins). Identical lines (clones) of these proliferate and cause elevations in blood proteins such as monoclonal (M) protein (an abnormal antibody or part of an antibody), resulting in fatigue and bone hardening.

Major criteria are:

  • Organomegaly: The liver, spleen, and lymph nodes can become enlarged. This can cause problems with how the organs function—leading to symptoms corresponding to the affected organs. Organomegaly can also cause the abdomen to appear large and swollen. 
  • Endocrinopathy/edema: The thyroid gland, adrenal glands, pituitary gland, and pancreas can swell and experience impaired function. Pancreatic disease can cause diabetes. Thyroid disease can cause hyperthyroidism or hypothyroidism. Pituitary disease can cause male breast enlargement or menstrual problems.
  • Skin changes: The skin can become red, dark, swollen, and thick in areas throughout the body. 

Polyneuropathy and monoclonal proteins are necessary features for a diagnosis of POEMS syndrome, and one or more of the other features are usually present as well. 

POEMS syndrome may also be known as:

  • Crow-Fukase syndrome
  • Takatsuki disease
  • PEP syndrome (plasma cell dyscrasia, endocrinopathy, polyneuropathy)
  • Osteosclerotic myeloma

What Causes POEMS?

People with POEMS syndrome have an increased number of plasma cells, which can cause damage to other parts of the body. There is no known trigger or underlying cause for POEMS syndrome, and it doesn’t follow a hereditary pattern. Symptoms begin during adulthood, but there are no known contributing factors.

The condition is considered to have autoimmune and paraneoplastic features. Autoimmune disorders are conditions that occur when the immune system attacks its own body tissues. Paraneoplastic conditions occur when cancer proteins induce tissue damage in the body.

Research suggests that excess numbers of clonal lines of plasma cells may lead to the accumulation of M protein in the blood. This is believed to trigger inflammation and may promote the release of excess growth factors in the blood that may contribute to organ enlargement.  

Vascular endothelial growth factor (VEGF), which promotes the growth of blood vessels, is also believed to contribute to some aspects of POEMS syndrome. Some treatments work to prevent the progression of the disease by targeting VEGF.

Other Symptoms of POEMS

The diverse symptoms of POEMS syndrome can develop at any time during the disease course, and they don’t have to follow a specific sequence. You might have some symptoms but not others.

Symptoms of POEMS can include:

  • Swelling in arms and legs: Monoclonal protein accumulation, plasma cell excess, endocrine disorders, or organ failure may cause swelling of the arms and legs.
  • Unexplained weight loss: Organ failure or endocrine disease can affect weight.
  • Bone and joint pain: Hardened areas of the bone can lead to pain and an increased risk of fractures.
  • Vision problems: Papilledema (swelling in the eyes) can cause vision loss.
  • Trouble breathing: Nerve disease can impair the muscles that control breathing.
  • Fatigue: Many of the effects of POEMS syndrome can cause fatigue. A few effects that may contribute to fatigue include thyroid disease, shortness of breath, and swelling.
  • Fever: Sometimes, elevated proteins or plasma cells in the blood can cause a fever.

Diagnostic Criteria for POEMS Syndrome

A diagnosis of POEMS syndrome requires the following:

  • Mandatory criteria: Polyneuropathy and monoclonal plasma cell proliferation
  • One or more of the major criteria: Castleman disease (a type of lymph node enlargement), osteosclerotic lesion, elevated VEGF
  • One or more of the minor criteria: Organomegaly, swelling in the body, endocrine disorder, skin changes, papilledema, thrombocytosis (a high number of platelets, which are blood cells that help you to stop bleeding)

How Is POEMS Diagnosed?

If you have POEMS syndrome, there’s a high likelihood that it wouldn’t be recognized when signs first appear. That’s because your symptoms and diagnostic testing would be directed to features you’re experiencing.

It’s not until you begin to develop several diverse effects that your healthcare providers would recognize the potential link among the symptoms you’re having.

It’s also possible to be misdiagnosed because signs and symptoms mimic those of other disorders. It may be initially diagnosed as multiple myeloma or another blood cell disorder.

If you develop polyneuropathy before any other symptoms, you might be diagnosed with Guillain-Barré syndrome or chronic inflammatory demyelinating polyneuropathy (CIDP), which are the most common types of polyneuropathy. Similarly, if you develop thyroid disease or diabetes, you would be diagnosed and treated for these endocrine disorders. 

POEMS syndrome is expected to progress rapidly and can be life-threatening without treatment. Diagnosis and treatment can help your outcome.

Some tests you can expect during the diagnostic process are:

  • Blood and urine tests: These tests may help evaluate the type and severity of organ failure. Results can guide the treatment of specific problems, such as adrenal gland disease. VEGF levels in the blood or urine can help identify POEMS syndrome, and inflammatory cells and proteins may also be present.
  • Imaging: Diagnostic imaging tests may identify hardened areas of bone or organomegaly, which can help differentiate POEMS syndrome from other disorders and can help direct treatment for specific effects of the disorder. 
  • Bone marrow biopsy: Monoclonal proteins can be detected with a bone marrow biopsy, which is an invasive diagnostic procedure. 
  • Electromyography: This test can identify nerve damage and can be useful in assessing polyneuropathy. 

Treatment for POEMS

There is no known cure for POEMS syndrome, but treatment may help to improve symptoms. Individualized treatments can alleviate specific effects, such as diabetes, thyroid disease, or pain from polyneuropathy. 

Disease-modifying treatments, such as the following, aim to control the plasma cell disorder:

  • Radiation therapy: Radiation therapy may be necessary as a treatment for plasma cell proliferation and bone disease. 
  • Chemotherapy: The chemotherapeutic medications used for treating POEMS syndrome may include a combination of cyclophosphamide, daratumumab, carfilzomib, pomalidomide, or elotuzumab. 
  • Stem cell transplant: This intervention is a replacement of cells in the bone marrow to treat the plasma cell disorder. 

Your healthcare team may also prescribe steroids or diuretics (water pills) to ease swelling.

What Happens If POEMS Syndrome Goes Untreated?

Without treatment, POEMS syndrome is expected to progress and cause more harm throughout the body. Damage to organs that are already affected may worsen, and additional areas of the body can begin to become involved as well. 

If you’ve been diagnosed with POEMS syndrome, it’s not possible to predict which features of the disorder you might develop in the future.  

Potential effects can include:

  • Diabetes: Dysregulation of blood sugar may cause dizziness, increased urination, kidney failure, vision loss, and diabetic nerve damage (in addition to the polyneuropathy of POEMS syndrome). 
  • Thyroid disease: High or low thyroid hormone levels can cause jitteriness, low energy, difficulty sleeping, and weight changes. 
  • Adrenal dysfunction: Blood pressure changes and swelling of the body can occur. 
  • Nerve damage: Weakness and difficulty walking may develop. 
  • Liver failure: Low energy and swelling are early signs. The body may become unable to process food and medication, which can cause brain damage. 

Eventually, without treatment, these effects can become life-threatening. 

How Long Can You Live With POEMS?

Living with POEMS syndrome is challenging due to the many health problems it causes. While some complications can be prevented or treated, the condition causes serious health issues that can be fatal.

After diagnosis, the median survival is 14 years, which means that about half of people with POEMS syndrome live longer than 14 years, and half of people with this disease do not survive as long as 14 years. 

It’s not possible to predict your life expectancy with certainty, but having treatable disease effects could mean that you are likely to survive for longer, while multiorgan failure may result in a shorter survival. 

Other Ways to Manage POEMS Syndrome

In addition to treatment with medication or procedures such as stem cell transplant, there are other nonmedical therapies that can help improve your quality of life when living with POEMS syndrome.

Lifestyle adjustments and nonmedical therapies you might need include:

  • Physical therapy: Interventions to help improve your mobility can provide you with strength and balance so that you can safely get around.
  • Diet: many types of organ failure and endocrine disorders can affect the way your body responds to certain foods. You may need to make some dietary adjustments. For example, if you have swelling, you may need to adjust your salt and fluid intake.
  • Pain control: Living with chronic pain can be extremely challenging. Some non-medical therapies that can help you cope may include massage, acupuncture, or exercise.
  • Mental health: It can be overwhelming to have a rare disorder that is not well understood. If you are feeling alone, stressed, or depressed, it may help to speak with a therapist about your feelings.

Complications Associated With POEMS

There are a number of different complications that can occur due to POEMS syndrome.

Sometimes complications may be life-threatening, including:

  • Fluid buildup in the abdomen or chest can cause difficulty breathing. 
  • Severe neuropathy may lead to injuries.
  • Bone thickening can lead to fractures.
  • Diabetes can cause kidney failure.

Is POEMS Syndrome the Same As Multiple Myeloma?

Multiple myeloma is a type of blood cancer characterized by plasma cell proliferation. It has some similarities to POEMS syndrome, but it is not the same. These diseases share some similar symptoms and blood test results.

A key difference between multiple myeloma and POEMS syndrome is that POEMS syndrome causes a demyelinating polyneuropathy, as well as effects on other organ systems in the body—this is not characteristic of multiple myeloma.

The chemotherapeutic treatments used for POEMS syndrome are similar to the treatments used for multiple myeloma, as the plasma cells in POEMS syndrome play a role in the damage caused by the condition.

When to Contact a Healthcare Provider

You must get medical attention if you experience any new or worsening symptoms. If you haven’t been diagnosed with POEMS syndrome, the symptoms could indicate POEMS syndrome or another disorder. You would need a diagnosis and prompt treatment.

If you’ve already been diagnosed with POEMS syndrome, new or worsening symptoms could indicate worsening organ damage or new organ damage.

Get medical attention if you have the following:

  • Weakness of the arms or legs
  • Swelling of your arms or legs
  • Enlargement in your neck or abdomen
  • Tingling, pain, or changes in sensation
  • Vision changes
  • Shortness of breath
  • Persistent fatigue
  • Unexplained weight loss

Summary

POEMS syndrome is a rare blood and nerve disorder that doesn't have a known cause or known risk factors. The features include plasma cell proliferation and nerve disease throughout the body, with additional diagnostic criteria. Diagnosis may be delayed because it's so rare and poorly understood.

Treatments can reduce some of the effects of POEMS syndrome, and some treatments may delay the disorder's progression. If you or a loved one is living with POEMS syndrome, you must seek medical care from a healthcare team with experience in this condition and learn about maintaining the best possible quality of life.

7 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
  1. National Organization for Rare Diseases. POEMS syndrome.

  2. Bou Zerdan M, George TI, Bunting ST, Chaulagain CP. Recent advances in the treatment and supportive care of POEMS syndrome. J Clin Med. 2022;11(23):7011. doi:10.3390/jcm11237011

  3. Khouri J, Nakashima M, Wong S. Update on the diagnosis and treatment of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome: A review. JAMA Oncol. 2021;7(9):1383–1391.doi:10.1001/jamaoncol.2021.0586

  4. Brioli A, Wyrwa A, Rüddel U, et al. Mutations in the plasma cell clone identify mechanism of polyneuropathy in a case of POEMS syndrome associated with Castleman disease and multiple myeloma. Ann Hematol. 2023;102(1):239-242. doi:10.1007/s00277-022-05032-1

  5. Oyenuga M, Mohamed MMG, Patel R, Sartaj S, Sen S, Lacasse A, Kumar V. POEMS Syndrome - a diagnostic dilemma with challenging presentation. J Community Hosp Intern Med Perspect. 2022;12(6):69-72. doi:10.55729/2000-9666.1121

  6. Ma J, Chen Y, Qin X, et al. POEMS syndrome: a rare cause of ascites and pelvic effusion. Clin Case Rep. 2022;10(11):e6603. doi:10.1002/ccr3.6603

  7. Vaxman I, Kumar SK, Buadi F, et al. Daratumumab, carfilzomib, and pomalidomide for the treatment of POEMS syndrome: The Mayo Clinic experience. Blood Cancer J. 2023;13(1):91. doi:10.1038/s41408-023-00859-x

Heidi Moawad, MD

By Heidi Moawad, MD
Heidi Moawad is a neurologist and expert in the field of brain health and neurological disorders. Dr. Moawad regularly writes and edits health and career content for medical books and publications.