POEMS Syndrome: A Rare Blood Disorder Affecting Nerves and Other Body Parts

Overview Types Causes & Risk Factors Symptoms Preventions Treatment

Overview

POEMS syndrome is a rare blood disorder that affects the nerves and other parts of the body. It is a type of plasma cell disorder that is characterized by the production of abnormal amounts of a protein in the bone marrow. This protein can cause damage to various parts of the body, including the nerves, skin, and organs.

Types of POEMS syndrome

There are no distinct subtypes of POEMS syndrome, but it can be classified based on the severity and extent of the disease. Mild cases may not require immediate treatment, while severe cases may require prompt medical attention.

Causes of POEMS syndrome

The exact cause of POEMS syndrome is not known, but it is believed to be related to an increase in the number of plasma cells in the bone marrow. These cells produce an abnormal amount of a protein called monoclonal-protein, which can cause damage to various parts of the body.

Risk Factors of POEMS syndrome

There are no known risk factors for developing POEMS syndrome, but people with certain conditions such as multiple myeloma or lymphoma may be more likely to develop it.

Symptoms of POEMS syndrome

The symptoms of POEMS syndrome can vary depending on the severity and extent of the disease. Common symptoms include:

1. Polyneuropathy: Numbness, tingling, and weakness in the legs and hands.

2. Organomegaly: Enlarged spleen, liver, or lymph nodes.

3. Endocrinopathy/edema: Abnormal hormone levels that can cause symptoms such as hypothyroidism, diabetes, sexual problems, fatigue, and swelling in the limbs.

4. Monoclonal-protein: Abnormal bone marrow cells that produce an abnormal amount of protein.

5. Skin Changes: Changes in skin color, red spots, thicker skin, and increased facial or leg hair.

6. Other Symptoms: Difficulty breathing, weight loss, and muscle weakness.

Preventions of POEMS syndrome

There is no known way to prevent POEMS syndrome, as it is a rare blood disorder that is caused by an abnormal increase in plasma cells in the bone marrow.

Diagnosis of POEMS syndrome

Diagnosis of POEMS syndrome typically involves:

Physical examination: A thorough physical examination to assess for signs of nerve damage or organ enlargement.
Blood tests: Blood tests to assess for abnormal hormone levels and to detect monoclonal-protein in the blood.
Imaging tests: Imaging tests such as X-rays or MRI scans to assess for signs of organ enlargement.
Bone marrow biopsy: A biopsy of the bone marrow to assess for abnormal plasma cells.

Treatment for POEMS syndrome

Treatment and management of POEMS syndrome typically involve:

Medications: Medications such as corticosteroids or chemotherapy to reduce the production of monoclonal-protein.
Supportive care: Supportive care such as rest and hydration to manage symptoms such as fatigue and swelling.
Physical therapy: Physical therapy to manage symptoms such as muscle weakness and joint pain.
Surgical intervention: Surgical intervention may be necessary to remove enlarged organs or repair damaged nerves.

When to seek medical attention ?

If you are experiencing symptoms such as numbness or tingling in your legs or hands, difficulty breathing, or weight loss, it is essential to see a doctor for proper evaluation and diagnosis.