Not Found
Locations

Find information on medical topics, symptoms, drugs, procedures, news and more, written for the health care professional.

POEMS Syndrome

(Crow-Fukase Syndrome; Takatsuki Disease; PEP Syndrome)

By Jennifer M. Barker, MD, Associate Professor of Pediatrics, Division of Pediatric Endocrinology, Children's Hospital Colorado, Division of Pediatric Endocrinology

Click here for
Patient Education

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) is a nonautoimmune polyglandular deficiency syndrome.

POEMS syndrome is probably caused by circulating immunoglobulins caused by a plasma cell dyscrasia. Circulating cytokines (IL-1-β, IL-6), vascular endothelial growth factor, and tumor necrosis factor-alpha are also increased.

Patients may have the following:

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).

Like other syndromes of undefined pathophysiology, POEMS syndrome is diagnosed based on the constellation of symptoms and signs. Criteria include the presence of polyneuropathy and monoclonal paraproteinemia plus any 2 of the other manifestations of the disorder.

Treatment

  • Radiation therapy

  • Chemotherapy with or without hematopoietic stem cell transplantation

Treatment of POEMS syndrome consists of radiation therapy or chemotherapy sometimes followed by autologous hematopoietic stem cell transplantation (1). Five-year survival is about 60%.

Treatment reference

  • 1. Dispenzieri A: POEMS syndrome: 2017 update on diagnosis, risk-stratification, and management. Am J Hematol 92:814–829, 2017. doi: 10.1002/ajh.24802