POEMS syndrome is a rare blood disease that affects multiple body systems, including the immune system, which results in abnormal cell production and irregular antibody behavior. The chronic condition can be life-threatening, but the symptoms can be managed with treatment, medication, and therapy.
POEMS syndrome is a paraneoplastic disorder, diseases marked by the immune system's abnormal response to a cancerous tumor. A healthy immune system produces proteins called antibodies in response to anything foreign in the bloodstream, including bacteria and viruses. In paraneoplastic disorders, however, white blood cells produce antibodies that attack healthy cells. Paraneoplastic disorders are most often comorbid with a malignant disease such as cancer.
This rare disease attacks the blood and nervous system, causing an overproduction of mature B lymphocytes or plasma cells, which results in the production of the antibody M protein. M proteins, which are generally not necessary for immune system function, attack healthy cells in the nervous system and damage multiple organ systems. The acronym POEMS relates to types of symptoms the condition causes:
People with POEMS syndrome often experience polyneuropathy or damage to the peripheral nervous system, which lies outside the central nervous system and relays sensory and motor information to and from the brain and spinal cord. Damage to these nerves results in disordered sensation and movement. People with polyneuropathy may experience numbness, tingling, and weakness that begins in the legs that, if untreated, travels upward to the arms and feet.
Organomegaly is enlargement of the internal organs; in cases of POEMS syndrome, the liver and spleen are most commonly affected. The disorder might also lead to swelling of the lymph nodes. Organomegaly often results in a host of symptoms, including abdominal pain, nausea, and fatigue.
POEMS syndrome might also lead to disturbances in the endocrine system, resulting in abnormal levels of hormones in the bloodstream. The development of diabetes is often the most common endocrinopathy. Hypothyroidism and poor functioning of the adrenal gland might also occur. Malfunctioning ovaries in females and testes in males is prevalent.
Monoclonal gammopathies are characterized by the overgrowth of plasma cells in the bones. Excess cells can develop into neoplasms, plasma-containing tumors in the bones, as well as sclerotic bone lesions. Plasma overgrowth also leads to the overproduction of the M protein, the antibody responsible for cell and organ damage in POEMS syndrome.
POEMS syndrome may lead to a host of skin abnormalities, such as hyperpigmentation (darkening) or thickening of the skin. Excessive sweating and hair growth, particularly on the face and legs, might also result. The condition can also cause the growth of blood vessels that leave deep red blotches on the skin, though this symptom is rare.
Experts are unsure why the immune system sometimes produces too many plasma cells and M protein antibodies. There is evidence of a link between POEMS syndrome and overproduction of vascular endothelial growth factor (VEGF). VEGF is instrumental in the development and repair of endothelium, the cells lining the blood vessels. A causal relationship between the syndrome and elevated cytokine levels might also exist. Cytokines are communicator cells that aid in immune and inflammatory responses.
If a doctor suspects POEMS syndrome, he or she will perform a physical exam, symptom check, and patient history. A series of diagnostic tests can confirm the disorder. They may include looking for elevated levels of certain hormones and proteins in the blood or urine and evidence of abnormal bone growth and enlarged organs through imaging tests. A bone tissue biopsy can find abnormal plasma cells. The presence of immunologic abnormalities confirms the diagnosis, including evidence of M proteins and VEGF.
Treating POEMS syndrome involves addressing the underlying paraneoplastic disorder and providing symptom relief. Chemotherapy or radiation therapy destroys the overabundant plasma cells. Medication can relieve swelling, and hormone replacement therapy improves endocrine abnormalities. Physical therapy may help manage the symptoms associated with polyneuropathy. With proper care and therapy, people with POEMS syndrome can lead normal, healthy lives.
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