POEMS Syndrome

Patients may have the following:

• Adrenal insufficiency (Addison disease)

• Diabetes mellitus type 2

• Hepatomegaly

• Hyperparathyroidism

• Hypogonadism

• Lymphadenopathy

• 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. Approximately 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).

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/or hypothyroidism)

• Extravascular volume overload (eg, ascites, peripheral edema, pleural effusion)

• Organomegaly

• Papilledema

• Skin changes

• Thrombocytosis or polycythemia

• Radiation therapy

• Chemotherapy with or without hematopoietic stem cell transplantation

Treatment of POEMS syndrome consists of radiation therapy for localized disease and chemotherapy for disseminated disease, including bone marrow involvement, sometimes followed by autologous hematopoietic stem cell transplantation (1, 2). Five-year survival is approximately 60%.