POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) is a nonautoimmune polyglandular deficiency syndrome.
POEMS syndrome is a rare paraneoplastic disorder and is probably caused by circulating immunoglobulins caused by a plasma cell disorder. Circulating cytokines (interleukin 1-beta, interleukin 6), vascular endothelial growth factor (VEGF), and tumor necrosis factor-alpha are also increased.
Symptoms and Signs of POEMS Syndrome
Patients may have the following:
Adrenal insufficiency (Addison disease)
Diabetes mellitus type 2
Hepatomegaly
Hypogonadism
Monoclonal gammopathy
Osteoclastic bone lesions
Polyneuropathy (peripheral motor and sensory manifestations)
Primary hypothyroidism
Skin abnormalities (eg, hyperpigmentation, dermal thickening, hirsutism, angiomas, hypertrichosis)
Other symptoms and signs of POEMS syndrome may include edema, ascites, pleural effusion, papilledema, and fever. About 15% of patients with POEMS syndrome have associated Castleman disease (a lymphoproliferative disorder, some forms of which are associated with infection by HIV or human herpesvirus 8).
Diagnosis of POEMS Syndrome
Like other syndromes of undefined pathophysiology, POEMS syndrome is diagnosed based on the constellation of symptoms, signs and laboratory findings.
Patients must have both mandatory criteria and ≥ 1 major and ≥ 1 minor criteria.
Mandatory criteria (both):
Polyneuropathy
Evidence of monoclonal plasma cell proliferation
Major criteria (≥ 1):
Castleman disease
Elevated level of vascular endothelial growth factor (VEGF)
Sclerotic bone lesions
Minor criteria (≥ 1):
Endocrinopathy (other than diabetes and hypothyroidism)
Extravascular volume overload (eg, ascites, peripheral edema, pleural effusion)
Organomegaly
Papilledema
Skin changes
Thrombocytosis or polycythemia
Treatment of POEMS Syndrome
Radiation therapy
Chemotherapy with or without hematopoietic stem cell transplantation
Treatment of POEMS syndrome consists of radiation therapy or chemotherapy for bony lesions or bone marrow involvement sometimes followed by autologous hematopoietic stem cell transplantation (1). Five-year survival is about 60%.
Treatment reference
1. Khouri J, Nakashima M, Wong S: Update on the diagnosis and treatment of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) syndrome. JAMA Oncol 7(9):1383-1391, 2021. doi: 10.1001/jamaoncol.2021.0586