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What is POEMS syndrome?
The acronym POEMS refers to
- • P olyneuropathy
- • O rganomegaly
- • E ndocrinopathy
- • M onoclonal protein
- • S kin involvement
POEMS syndrome stands for:
- • P olyneuropathy: a demyelinating sensorimotor peripheral neuropathy is seen in 100% of patients. At least 50% of patients develop severe muscle weakness.
- • O rganomegaly: hepatomegaly, splenomegaly, or lymphadenopathy develops in 50%.
- • E ndocrinopathy: 67% have or develop at least one endocrine abnormality. The most common is hypogonadism. Abnormalities of the adrenal and pituitary gland can occur. DM and hypothyroidism also can be present but do not count as criteria due to their frequency in the general population.
- • M onoclonal proteins: 100% have a monoclonal protein in the serum and/or urine. It is almost always associated with a lambda light chain.
- • S kin changes: occur in 67% of patients. Hyperpigmentation and hemangiomas are most common (40%–50%). Hypertrichosis is also common. Scleroderma-like changes with Raynaud’s or acrocyanosis can also be seen (20%–30%).
- • There are numerous other manifestations such as extravascular volume overload (edema, ascites, etc.), hematologic abnormalities, papilledema, as well as others.
POEMS syndrome is an expanded variant of osteosclerotic myeloma with peripheral neuropathy.
4 Interesting Facts of POEMS syndrome
- Plasma cell disorder characterized by the rare constellation of findings of polyneuropathy, organomegaly, endocrinopathy, elevated monoclonal immunoglobulin level, and skin changes
- Monoclonal proliferation of plasma cells does occur in POEMS syndrome, but at a lower level than in multiple myeloma
- Usually presents with a single osteolytic bone lesion
- Imaging findings of POEMS syndrome include organomegaly and sclerotic osseous lesions that display the same fludeoxyglucose F 18 avidity as normal bone
- Osteosclerotic aspect of the bone lesions distinguishes POEMS syndrome from multiple myeloma and solitary plasmacytoma
Not all features of the syndrome are required to make the diagnosis.
POEMS is a very rare disease and requires a monoclonal plasma cell disorder with a progressive sensorimotor peripheral neuropathy and at least one other key criterion, particularly the presence of Castleman’s disease (angiofollicular lymph node hyperplasia) or osteosclerotic myeloma. In general, kidney involvement is not a usual feature, but when kidney involvement is present, it is unrelated to LC deposition. Definitive therapy is uncertain but may involve radiotherapy and chemotherapy regimens directed toward the myeloma component.
POEMS syndrome occurs in middle-aged patients with a slight male predominance. It is commonly associated with osteosclerotic bone lesions (97%) and has been called osteosclerotic myeloma. Multicentric Castleman’s disease is seen in 15% to 20% of patients who have POEMS syndrome. This is notable since proinflammatory cytokines (IL-1, TNF, and IL-6) are elevated in both diseases. However, patients with POEMS also have significantly (more than three to four times upper limit of normal) elevated levels of vascular endothelial growth factor that is produced by platelets and plasma cells. This may contribute to some of the clinical manifestations. Patients with disseminated disease are treated similar to patients with multiple myeloma.
Patients typically have a chronic progressive sensory-motor demyelinating polyneuropathy, monoclonal gammopathy (usually lambda light chain), peripheral edema, ascites, hypertrichosis, diffuse hyperpigmentation and thickening of the skin, hepatomegaly, splenomegaly, lymphadenopathy, gynecomastia, impotence, amenorrhea, and digital clubbing. Increased serum level of vascular endothelial growth factor (VEGF) is frequently observed in POEMS syndrome.
Treatment results in a decrease in the level of VEGF, which correlates with symptomatic improvement, though there can be a delayed response in VEGF levels.
Sources
- Mehndiratta MM, Hughes RA: Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev (9):CD003906, 2012.
- Isose S, Mori M, Misawa S, et al: Long-term regular plasmapheresis as a maintenance treatment for chronic inflammatory demyelinating polyneuropathy. J Peripher Nerv Syst 15:147-149, 2010.
- Hahn AF, Bolton CF, Pillay N, et al: Plasma exchange therapy in chronic inflammatory demyelinating polyneuropathy: A double-blind, sham-controlled, cross-over study. Brain 119:1055-1066, 1996.
- Hughes RA, Donofrio P, Bril V, et al: Intravenous immune globulin (10% caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): A randomised placebo-controlled trial. Lancet Neurol 7:136-144, 2008.
- Eftimov F, Winer JB, Vermeulen M, et al: Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev (12):CD001797, 2013.
- Nobile-Orazio E, Gallia F: Multifocal motor neuropathy: Current therapies and novel strategies. Drugs 73(5):397-406, 2013.
- Olney RK, Lewis RA, Putnam TD, et al: Consensus criteria for the diagnosis of multifocal motor neuropathy. Muscle Nerve 27:117-121, 2003.
- Slee M, Selvan A, Donaghy M: Multifocal motor neuropathy: The diagnostic spectrum and response to treatment. Neurology 69:1680-1687, 2007.
