What is POEMS syndrome?
POEMS syndrome is an extremely rare blood disorder that affects several body systems. Its name is a combination of the names of each system it affects and the symptoms patients may experience.
Polyneuropathy describes a breakdown of the motor and sensory nerves that connect the brain and spinal cord to the rest of the body. POEMS syndrome patients experience numbness, tingling, and weakness in the toes, feet, and later the hands.
Organomegaly refers to enlargement of the liver and spleen and swelling of the lymph nodes.
Endocrinopathy points to problems with the endocrine system, the glands that secrete hormones into the bloodstream. POEMS syndrome patients may have an underactive thyroid, metabolism issues, diabetes, or improper functioning of the ovaries or testes.
Monoclonal protein is a signal there may be uncontrolled growth of abnormal plasma cells in the blood. POEMS syndrome patients may experience tumors known as plasmacytomas that appear on x-rays as thickening of the bone.
Skin abnormalities may include darkening of the skin, hardening or thickening of the skin, increased facial or leg hair, and excessive sweating.
As the condition progresses it can lead to problems in the lungs, kidneys, and heart.
How POEMS syndrome is detected
Once doctors gather a medical history and conduct a physical exam, they will order blood and urine tests to check protein and hormone levels. One sign they look for is elevated vascular endothelial growth factor (VEGF), a substance that helps a cancer grow new blood vessels.
Because POEMS syndrome affects several systems, the tests to detect it may include:
- Body imaging, like CT and PET, to check for unusual bone growth and enlargement of organs
- Tissue samples (biopsies) of bone or bone marrow to check for abnormal plasma cells
- Lymph node samples to test for other diseases that often parallel POEMS syndrome
- Lung, heart, nerve, and endocrine tests to check on normal functioning
Treating POEMS syndrome
Doctors take a two-pronged approach to treatment. First, they target the “M” in POEMS—the growth of abnormal plasma cells in the blood—as the underlying cause of the disease. Second, they work to relieve the symptoms caused by the disease.
Each patient’s case is different, and doctors will choose a course of treatment that shows the greatest promise for effective results for their individual patient.
Dramatic improvements have been demonstrated through stem cell transplant. Doctors will use high doses of radiation to wipe out a patient’s stem cells and then reset the patient’s stem cells by reintroducing the patients’ own healthy cells to the bone marrow. This gives the body a fresh start in producing its own agents to destroy the rapidly reproducing cancer cells.
Other treatments to target the abnormal plasma cells may include:
- Chemotherapy
- Radiation therapy
- Monoclonal antibody therapy
- Treatments addressing other POEMS syndrome symptoms include
- Physical therapy to strengthen legs and combat the effects of neuropathy
- Diuretics to decrease excess fluid and swelling
- Hormone replacement to address endocrine abnormalities
What to expect following treatment for POEMS syndrome
POEMS syndrome patients can be effectively treated and return to an overall normal quality of life. The key for addressing the many facets of POEMS syndrome is being cared for by a team of specialists, including myeloma specialists, radiologists, neurologists, nephrologists, cardiologists, physical therapists, pharmacists, and others.