POEMS syndrome

POEMS syndrome
Other names	Crow–Fukase syndrome, Osteosclerotic myeloma, PEP syndrome, Polyneuropathy-endocrinopathy-plasma cell dyscrasia syndrome, Takatsuki syndrome.[1]
Multiple myeloma is usually diagnosed because malignant plasma cells continue to produce an antibody that can be detected as a paraprotein.
Specialty	Oncology
Symptoms	Papilledema,[2] polyneuropathy,[1] edema,[3][4] hyperpigmentation, hypogonadism,[1] visceromegaly,[1] amenorrhea,[3][4] gynecomastia,[3][4] erectile dysfunction,[3][4] and testicular atrophy.[3][4]
Complications	Multiorgan failure, restrictive lung disease, pulmonary hypertension, cardiorespiratory failure, progressive inanition, infection, capillary leak-like syndrome, and renal failure.[5]
Usual onset	5th to 6th decade of life.[6]
Duration	Chronic.[6]
Diagnostic method	Clinical Criteria.[6]
Differential diagnosis	Chronic inflammatory demyelinating polyradiculoneuropathy, cryoglobulinemia, nodular localized cutaneous amyloidosis, multiple myeloma, monoclonal gammopathy of undetermined significance, waldenstrom hypergammaglobulinemia, scleroderma, raynaud phenomenon, amyloid light chains (AL) amyloidosis, guillain-Barré syndrome.[6][7]
Treatment	Corticosteroids, low-dose alkylators, peripheral blood stem cell transplantation, high-dose chemotherapy.[6]
Medication	Thalidomide, lenalidomide, bortezomib, bevacizumab.[5]
Prognosis	Median survival of 13.8 years.[8]
Frequency	Rare

POEMS syndrome (also termed osteosclerotic myeloma, Crow–Fukase syndrome, Takatsuki disease, or PEP syndrome) is a rare paraneoplastic syndrome caused by a clone of aberrant plasma cells. The name POEMS is an acronym for some of the disease's major signs and symptoms (polyneuropathy, organomegaly, endocrinopathy, myeloma protein, and skin changes), as is PEP (polyneuropathy, endocrinopathy, plasma cell dyscrasia).

The signs and symptoms of most neoplasms are due to their mass effects caused by the invasion and destruction of tissues by the neoplasms' cells. Signs and symptoms of a cancer causing a paraneoplastic syndrome result from the release of humoral factors such as hormones, cytokines, or immunoglobulins by the syndrome's neoplastic cells and/or the response of the immune system to the neoplasm. Many of the signs and symptoms in POEMS syndrome are due at least in part to the release of an aberrant immunoglobulin, i.e. a myeloma protein, as well as certain cytokines by the malignant plasma cells.^[9][3][4]

POEMS syndrome typically begins in middle age – the average age at onset is 50 – and affects up to twice as many men as women.