POEMS syndrome

Symptoms and causes

POEMS syndrome is a rare blood disorder that damages nerves and affects other parts of the body. POEMS stands for symptoms of the condition. Symptoms may include weak legs, trouble breathing, a larger spleen or liver, and skin changes.

Without treatment, POEMS syndrome gets worse quickly. It can become life-threatening. So early diagnosis is vital.

There's no cure for POEMS syndrome. But treatment might improve symptoms. Treatment may include radiation therapy, chemotherapy and stem cell transplant. The outlook for people with POEMS syndrome has improved greatly over the years.

The symptoms of POEMS syndrome make up its name. Symptoms most often appear over weeks to months. They include the following:

• Polyneuropathy. This may include numbness, tingling and weakness in the legs. In time, it might affect the hands. It also involves trouble breathing. Polyneuropathy must be present for a POEMS diagnosis.
• Organomegaly. This is an enlarged spleen, liver or lymph nodes.
• Endocrinopathy and edema. Atypical hormone levels can cause an underactive thyroid, also called hypothyroidism. The hormone levels also can cause diabetes, sexual issues, tiredness, and swelling in arms and legs.
• Monoclonal protein. This is a protein that atypical plasma cells in bone marrow make. The monoclonal protein goes into the bloodstream. This must be present for the diagnosis of POEMS syndrome. Monoclonal protein often is linked with bones getting harder or thicker.
• Skin changes. This may involve having more color than usual on the skin. There may be red spots, which may be harder to see on Black or brown skin. The skin might be thicker. There might be more hair on the face or the legs.

Not all the symptoms of POEMS syndrome are in the name. And not everyone with POEMS syndrome has all the symptoms in the name. But everyone with POEMS syndrome has polyneuropathy and monoclonal protein.

Other symptoms some people with POEMS syndrome have spell out PEST. PEST stands for:

• Papilledema. This is swelling in the back of the eyes.
• Extravascular volume overload. This might include ankle swelling, fluid between the lung and the ribs, or fluid in the belly.
• Sclerotic bone lesions. X-rays can show these bone spots. They're most often not painful.
• Thrombocytosis and erythrocytosis. These are higher levels of red cells and platelets in the bloodstream.

Experts don't know the cause of POEMS syndrome. But people with POEMS syndrome have a higher number of plasma cells. These cells make too much of a type of protein, called monoclonal protein. This protein can damage other parts of the body.

Factors that raise the risk of POEMS syndrome include:

• Older age. POEMS syndrome most often happens after age 50.
• Male sex. People assigned male at birth are a bit more likely to get POEMS syndrome than are people assigned female at birth.