Poems Syndrome – Symptoms and Causes
Overview
POEMS syndrome is a rare blood condition that affects the nerves and multiple organ systems.
Medical experts haven’t identified the exact cause of POEMS syndrome. However, they know it involves an increase in plasma cells that produce harmful proteins, damaging various body systems.
The condition is often misdiagnosed because its symptoms resemble other disorders. Without treatment, POEMS syndrome progresses quickly and can become fatal, making early diagnosis crucial.
Symptoms
POEMS syndrome is a rare condition with a wide range of symptoms that affect multiple systems in the body. The name “POEMS” is an acronym representing five key features of the disease: Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, and Skin changes.
Polyneuropathy
This is often the first symptom to appear and is required for diagnosis. It involves nerve damage that leads to numbness, tingling, and muscle weakness.
Symptoms typically begin in the feet and progress upward, making walking and balance increasingly difficult. In advanced cases, the hands and even respiratory muscles can become involved.
Organomegaly
This refers to the enlargement of internal organs, particularly the liver, spleen, and lymph nodes. These changes may be noticeable as swelling in the abdomen or neck.
Endocrinopathy
Hormonal imbalances are common in POEMS syndrome and may include:
- Diabetes
- Thyroid disorders such as hypothyroidism
- Low testosterone levels in men
- Irregular menstrual periods in women
- Adrenal gland dysfunction
- Sexual dysfunction and metabolic disturbances
Monoclonal Protein
This involves the presence of an abnormal protein produced by plasma cells in the bone marrow. Itโs a critical diagnostic marker and is often associated with unusual bone hardening or lesions seen on imaging studies.
Skin Changes
A variety of skin abnormalities may occur, including:
- Darkening or hyperpigmentation
- Thickening of the skin
- Increased hair growth on the face or legs
- Whitened fingernails
- Small red or purple spots under the skin
Additional Symptoms
Other common features of POEMS syndrome include:
- Swelling in the limbs (edema)
- Fluid around the lungs (pleural effusion)
- Fatigue
- Unexplained weight loss
- Fever without infection
Due to its complex nature, the symptoms of POEMS syndrome vary between individuals and often worsen over time without treatment.
Causes
POEMS syndrome happens when plasma cells in the bone marrow grow abnormally. These cells make too much of a protein called monoclonal protein or M protein. Scientists don’t fully understand why these cells start growing out of control.
Plasma cells are part of the immune system. They normally help fight infections by making antibodies. In POEMS syndrome, these cells make harmful proteins that damage nerves, organs, and tissues.
Genetic factors might play a role, but POEMS syndrome is not typically inherited. Most cases appear randomly without a family history of the condition.
Some research suggests that exposure to certain viruses or toxins might trigger the disease in people who are already at risk.
Risk Factors
Several factors may increase a person’s chance of developing POEMS syndrome.
Age plays a significant role. Most cases are diagnosed in people between 50 and 70 years old.
Men face a higher risk than women. Studies show that POEMS syndrome affects men about 2.5 times more often than women.
Japanese and Chinese ancestry may also increase risk. Research indicates higher rates of POEMS syndrome in these populations compared to others. However, the condition can affect people of any ethnic background.
People with a history of plasma cell disorders face a greater risk. These disorders include conditions like multiple myeloma and monoclonal gammopathy of undetermined significance (MGUS).
Genetic factors might contribute to risk, though researchers haven’t identified specific genes linked to POEMS syndrome. Some evidence suggests certain environmental factors, such as radiation exposure or specific viral infections, might play a role in some cases.
One or more risk factors don’t guarantee developing POEMS syndrome. Many people with these risk factors never develop the condition, while some people develop it without any known risk factors.
