Poems syndrome

POEMS syndrome is a rare paraneoplastic syndrome characterized by the presence of a plasma cell disorder and a combination of clinical features. The acronym POEMS stands for these core features: Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, and Skin changes.

Polyneuropathy

Polyneuropathy in POEMS syndrome is typically a bilateral, symmetric, progressive sensorimotor neuropathy. It often begins in the lower extremities and subsequently involves the upper extremities. The neuropathy is usually of the demyelinating type, which is characterized by damage to the myelin sheath that covers nerve fibers.

Organomegaly

Organomegaly refers to the enlargement of organs. In POEMS syndrome, this can include hepatomegaly (enlargement of the liver), splenomegaly (enlargement of the spleen), and/or lymphadenopathy (enlargement of the lymph nodes).

Endocrinopathy

Endocrinopathy involves the dysfunction of one or more endocrine glands. In POEMS syndrome, this can manifest as diabetes mellitus, hypothyroidism, hypogonadism, or adrenal insufficiency.

Monoclonal protein

The presence of a monoclonal protein (or M protein) is a key feature of POEMS syndrome. This is a protein produced in excess by an abnormal clone of plasma cells, often resulting in a monoclonal gammopathy.

Skin changes

Skin changes in POEMS syndrome can be diverse, including hyperpigmentation, hypertrichosis, hemangiomas, and white nails.

Diagnosis

The diagnosis of POEMS syndrome is based on the presence of both mandatory criteria (polyneuropathy and monoclonal plasma cell disorder), at least one of the three other major criteria (Castleman disease, sclerotic bone lesions, or elevated vascular endothelial growth factor), and at least one of the six minor criteria (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, or thrombocytosis/polycythemia).

Treatment

Treatment of POEMS syndrome is directed at the underlying plasma cell disorder. This can involve radiation therapy for localized disease, systemic therapy for disseminated disease, and supportive care for the management of symptoms.

NIH genetic and rare disease info

• NIH info on Poems syndrome

Poems syndrome is a rare disease.

Poems syndrome Resources
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