What is POEMS syndrome

POEMS syndrome is a rare disorder that affects multiple systems in the body. The acronym stands for Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes. It is a paraneoplastic syndrome, meaning it is associated with an underlying plasma cell disorder, typically multiple myeloma.

Polyneuropathy refers to the damage or dysfunction of peripheral nerves, leading to symptoms like numbness, tingling, weakness, and impaired motor function. Organomegaly involves the enlargement of organs such as the liver, spleen, or lymph nodes. Endocrinopathy refers to hormonal imbalances that can manifest as diabetes, thyroid dysfunction, or adrenal insufficiency.

Monoclonal gammopathy indicates the presence of an abnormal protein (monoclonal gammopathy of undetermined significance or a monoclonal protein) in the blood. Lastly, skin changes can include hyperpigmentation, thickening, or excessive hair growth.

POEMS syndrome is diagnosed based on the presence of at least two major criteria (polyneuropathy and monoclonal gammopathy) along with one minor criterion (organomegaly, endocrinopathy, or skin changes). Treatment involves addressing the underlying plasma cell disorder, managing symptoms, and providing supportive care.