Table of ContentsView AllTable of ContentsWhat Is It?CausesSymptomsDiagnosisTreatmentIf UntreatedLife ExpectancyManagementComplicationsMultiple Myeloma vs. POEMSWhen to See a Provider
Table of ContentsView All
View All
Table of Contents
What Is It?
Causes
Symptoms
Diagnosis
Treatment
If Untreated
Life Expectancy
Management
Complications
Multiple Myeloma vs. POEMS
When to See a Provider
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.
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What Is POEMS Syndrome?
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:
Major criteria are:
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 syndromeTakatsuki diseasePEP syndrome (plasma cell dyscrasia, endocrinopathy, polyneuropathy)Osteosclerotic myeloma
POEMS syndrome may also be known as:
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 andparaneoplasticfeatures.Autoimmune disordersare conditions that occur when the immune system attacks its own body tissues.Paraneoplastic conditionsoccur 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.
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:
Diagnostic Criteria for POEMS SyndromeA diagnosis of POEMS syndrome requires the following:Mandatory criteria: Polyneuropathy and monoclonal plasma cell proliferationOne or more of the major criteria: Castleman disease (a type of lymph node enlargement), osteosclerotic lesion, elevated VEGFOne or more of the minor criteria: Organomegaly, swelling in the body, endocrine disorder, skin changes, papilledema,thrombocytosis(a high number ofplatelets, which are blood cells that help you to stop bleeding)
Diagnostic Criteria for POEMS Syndrome
A diagnosis of POEMS syndrome requires the following:Mandatory criteria: Polyneuropathy and monoclonal plasma cell proliferationOne or more of the major criteria: Castleman disease (a type of lymph node enlargement), osteosclerotic lesion, elevated VEGFOne or more of the minor criteria: Organomegaly, swelling in the body, endocrine disorder, skin changes, papilledema,thrombocytosis(a high number ofplatelets, which are blood cells that help you to stop bleeding)
A diagnosis of POEMS syndrome requires the following:
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 asmultiple myelomaor another blood cell disorder.
If you develop polyneuropathy before any other symptoms, you might be diagnosed withGuillain-Barré syndromeor 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:
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:
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:
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:
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:
Is POEMS Syndrome the Same As Multiple Myeloma?
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.
Causes and Risk Factors of Multiple Myeloma
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:
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 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.National Organization for Rare Diseases.POEMS syndrome.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/jcm11237011Khouri 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.2021.0586Brioli 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/s00277-022-05032-1Oyenuga 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:10.55729/2000-9666.1121Ma 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/ccr3.6603Vaxman 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
7 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.National Organization for Rare Diseases.POEMS syndrome.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/jcm11237011Khouri 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.2021.0586Brioli 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/s00277-022-05032-1Oyenuga 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:10.55729/2000-9666.1121Ma 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/ccr3.6603Vaxman 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
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
National Organization for Rare Diseases.POEMS syndrome.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/jcm11237011Khouri 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.2021.0586Brioli 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/s00277-022-05032-1Oyenuga 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:10.55729/2000-9666.1121Ma 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/ccr3.6603Vaxman 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
National Organization for Rare Diseases.POEMS syndrome.
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
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
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
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
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
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
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