POEMS syndrome

The mandatory criteria for diagnosis of POEMS syndrome are a chronic progressive polyneuropathy and a monoclonal plasma cell proliferative disorder. There is usually an associated M protein of either IgG, IgA type, with lambda light chain restriction in all cases.

Two thirds of patients with lymphadenopathy have changes consistent with the plasma cell variant of Castleman disease. Osterosclerotic bone lesions are present in > 95% of cases. Organomegaly, primary hepatomegaly or splenomegaly, is present in at least half of patients. Skin changes occur in more than two thirds of cases.

Crow-Fukase syndrome

Osterosclerotic myeloma

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin

POEMS syndrome is a paraneoplastic syndrome associated with a plasma cell neoplasm, usually characterized by fibrosis and osteroslcerotic changes in bone trabecula, and often with lymph node changes resembling the plasma cell variant of Castleman disease.

The POEMS acronym stands for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes, but these components are not all required for diagnosis.

Arthralgias

Bone pain

Chronic progressive polyneuropathy

Clubbing

Edema

Endocrinopathy

Fatigue

Organomegaly

Papilledema

Radiographic bone abnormalities

Serous cavity effusions

Skin changes

Thrombocytosis

Weight loss

Median overall survival is 14.7 years

Most common causes of death are cardiorespiratory failure and infection

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 256-257

National Cancer Institute
Section: General Information About Plasma Cell Neoplasms
Pages: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq