What Is POEMS Syndrome?

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. This syndrome is considered rare,

POEMS Syndrome Signs and Symptoms

POEMS syndrome is characterized by 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 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, can cause enlargement of the abdomen, or can be visualized with imaging tests.
• Endocrinopathy: Changes in hormone production can manifest as diabetes, impotence, amenorrhea (stopped menstrual periods), hypothyroidism (low thyroid levels), and/or gynecomastia (breast development in males).
• Monoclonal gammopathy or monoclonal plasma proliferative disorder: Both disorders cause abnormal blood protein, which is present in all patients with POEMS syndrome.
• Skin changes: These include hyperpigmentation (dark areas of the skin); hypertrichosis (increased body hair) on the face, limbs, and chest; skin thickening and tightening; edema (swelling) 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
• Hyperparathyroidism (high levels of parathyroid hormone)
• Addison's disease (underactive adrenal glands)

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 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.

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 at least 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 healthcare provider will order tests, including:

• A complete blood cell count (CBC)
• Serum protein immunoelectrophoresis
• Urinalysis
• Thyroid hormone levels
• blood glucose levels
• Estrogen levels
• Bone X-rays
• A bone marrow biopsy

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 POEMS syndrome is so rare and includes such a wide variety of signs and symptoms, 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 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
• Stem cell transplant
• Mobility aids
• Physical therapy

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 after diagnosis.

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.