What is POEMS syndrome
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) – POEMS syndrome is a rare autoimmune related blood disorder that damages your nerves and affects many other parts of the body.
POEMS syndrome can be misdiagnosed because the signs and symptoms mimic those of other disorders. POEMS syndrome progresses rapidly and may become life-threatening, so early diagnosis is important.
POEMS stands for these signs and symptoms:
Polyneuropathy. Numbness, tingling and weakness in your legs — and over time, maybe in your hands — and difficulty breathing.
Organomegaly. Enlarged spleen, liver or lymph nodes.
Endocrinopathy. Abnormal hormone levels that can result in underactive thyroid (hypothyroidism), diabetes, sexual problems, fatigue, swelling in your limbs, and problems with metabolism and other essential functions.
Monoclonal plasma-proliferative disorder. Abnormal bone marrow cells (plasma cells) that produce a protein (monoclonal protein) that can be found in the bloodstream.
Skin changes. More color than normal on your skin, possibly thicker skin and increased facial or leg hair.
POEMS syndrome is a rare, mulitisystem disorder. POEMS stands for the disorder’s features, which may include Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes. Signs and symptoms may include progressive sensorimotor polyneuropathy; enlarged liver, spleen, and/or lymph nodes; a disorder of the endocrine glands (often with multiple abnormalities); a monoclonal plasma cell proliferative disorder; and darkening of the skin (hyperpigmentation). Various other symptoms may also occur and may vary among affected people. The underlying cause of the disorder is not well understood. POEMS syndrome is a chronic disorder, with a median survival time of 8-14 years. There is no standard treatment; management depends on the underlying plasma cell disorder and may include radiation therapy, chemotherapy, and/or hematopoietic cell transplantation.
The cause of POEMS syndrome is not well understood. POEMS syndrome is a paraneoplastic disorder, which means that the signs and symptoms of the syndrome appear as a manifestation of the plasma cell disorder that the patient has (most patients are seen with osteosclerotic myeloma or monoclonal gammopathy of unknown significance); however, the mechanism by which this occurs is unknown. It is associated with a chronic overproduction of some substances known as pro-inflammatory cytokines (small molecules that act as messengers between cells to promote inflammation). These include interleukins (IL-1b, IL6) which are cytokines that help regulate immune responses, TNFα (Tumor Necrosis Factor alfa is a cytokine which is involved in the inflammatory process) and vascular endothelial growth factor (VEGF – a powerful vasodilator that may cause leaky blood vessels) which seem to be important in this disorder.
This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
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