What Is POEMS Syndrome?

Laboratory technician holding test tubes containing blood samples
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In This Article

POEMS syndrome is a rare and serious blood disorder that affects many systems of the body. POEMS is an acronym that stands for the five clinical features that are the hallmarks of the disease: Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes.

POEMS is more common in men than women and usually affects people in their 40s and 50s, although it may occur at any age. It is not known exactly how often the syndrome occurs because it is so rare and difficult to diagnose correctly.

Other names for POEMS syndrome include:

  • Crow-Fukase syndrome
  • Takatsuki disease
  • Osteosclerotic myeloma

POEMS Syndrome Signs and Symptoms

POEMS syndrome is characterized by most or more of a cluster of five disorders that comprise the acronym for which the condition is named. In addition, there are several other clinical features that can with POEMS.

Primary symptoms of POEMS can include:

  • Polyneuropathy: The most common manifestation of POEMS, which involves nerve symptoms such as numbness, tingling, and/or weakness of the hands and feet
  • Organomegaly: Enlarged organs—usually the liver, spleen, or lymph nodes
  • Endocrinopathy: Changes in hormone production that can manifest as diabetes, impotence, stopped menstrual periods (amenorrhea), low thyroid levels (hypothyroidism), and/or breast development in males (gynecomastia)
  • Monoclonal gammopathy or monoclonal plasma proliferative disorder: Both disorders cause abnormal blood protein cells, a symptom that is present in all patients with POEMS syndrome.
  • Skin changes: These include increased skin pigment (hyperpigmentation); increased body hair (hypertrichosis) on the face, limbs, and chest; skin thickening and tightening; swelling (edema) of the legs and feet; and whitening of the nails. Skin changes occur in 50% to 90% of people with PEOMS syndrome.

Other signs that might be found during an exam or on an imagining test include:

  • Papilledema (swelling around the optic nerve)
  • Pleural effusion (fluid around the lungs)
  • Osteosclerosis (painless scars that show up on a bone X-ray)
  • Clubbing (enlargement of the fingertips)
  • An elevated platelet count
  • Swollen lymph glands
  • High levels of parathyroid hormone (hyperparathyroidism)
  • Underactive adrenal glands (Addison's disease)

Causes

The cause of POEMS syndrome is unknown. People with the disorder have an overgrowth of plasma cells and increased blood levels of vascular endothelial growth factor (VEGF), a protein produced by cells that stimulates the formation of blood vessels. Levels of chemicals in the blood called cytokines— interleukin-6, interleukin-1 and TNF-alpha—have also shown to be elevated.

POEMS and Co-occurring Disorders

Approximately 70% of people with POEMS experience improper function of the ovaries or testes, a condition known as primary gonadal failure.

As many as 50% of POEMS patients will develop diabetes.

About 15% of people with POEMS syndrome also have Castleman disease, in which there is an abnormal overgrowth of cells in the lymph system.

Diagnosis

In order for a diagnosis of POEMS to be made, the following criteria must be met:

  • The presence of both polyneuropathy and monoclonal gammopathy
  • The presence of one of the following: sclerotic (scarring) bone lesions, Castleman disease, or elevation in VEGF levels
  • The presence of one of the following: organomegaly, extravascular volume overload (edema, pleural effusion, or ascites), endocrinopathy, skin changes, papilledema, or thrombocytosis/polycythemia

In addition to taking a medical history and performing a physical exam, your doctor will order tests, including:

  • A complete blood cell count (CBC)
  • Serum protein immunoelectrophoresis
  • Urinalysis
  • Testing of thyroid hormone levels
  • Testing of blood glucose levels
  • Testing of estrogen levels
  • A bone marrow biopsy and/or bone x-rays

Other diagnostic tests may include:

  • An eye exam
  • A neurologic exam
  • An examination of the skin
  • Imaging tests for heart disease
  • Evaluations for edema
  • Evaluations for pleural effusion and pericardial effusion
  • Measurement of serum or plasma levels

Differential diagnosis

Because many physicians are not familiar with POEMS syndrome, the condition is commonly misdiagnosed. The most common conditions that should be ruled out before arriving at a POEMS diagnosis are multiple myeloma, chronic inflammatory demyelinating polyneuropathy, AL amyloidosis, Guillain-Barré syndrome, and monoclonal gammopathy of undetermined significance (MGUS).

Treatment

Specialists such as a neurologist, hematologist, dermatologist, and an endocrinologist are usually involved in the care of those with POEMS syndrome.

Treatments vary according to the specific symptoms, underlying causes, and co-occurring disorders, and can include:

  • Radiation therapy
  • Corticosteroid medications such as prednisone
  • Immunotherapy
  • Chemotherapy
  • Hormone replacement therapy
  • Physical therapy
  • Bone marrow transplant
  • Mobility aids
  • Supplemental oxygen
  • Diabetes medications

Prognosis

POEMS syndrome is a chronic condition that can result in permanent disability or death. The overall median survival has been estimated to be 13.7 years.

The most common causes of death in POEMS patients are cardiorespiratory failure, renal failure, infection, and malnourishment. 

A Word From Verywell

Having a rare condition such as POEMS syndrome can be very isolating, but several organizations are committed to educating, advocating for, and providing support for people with rare diseases, including the Genetic and Rare Disease Information Center (GARD) and the National Organization for Rare Disorders (NORD). In addition to offering a variety of resources for people with rare diseases, these organizations can help you find clinical trials that may be researching treatments for POEMS.

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Article 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 Disorders. POEMS Syndrome.

  2. Dispenzieri, A. POEMS syndrome: 2017 Update on diagnosis, risk stratification, and management. American Journal of Hematology. 12 July 2017. doi:10.1002/ajh.24802

  3. Merck Manual. Professional Version. POEMS Syndrome.

  4. Genetic and Rare Diseases Information Center (GARD). POEMS Syndrome.

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