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POEMS Syndrome

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

By

Jennifer M. Barker

, MD, Children's Hospital Colorado, Division of Pediatric Endocrinology

Last full review/revision Jun 2019| Content last modified Jun 2019
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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 disorder. Circulating cytokines (interleukin 1-beta, interleukin 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 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. Dispenzieri A: POEMS syndrome: 2017 update on diagnosis, risk-stratification, and management. Am J Hematol 92:814–829, 2017. doi: 10.1002/ajh.24802

Click here for Patient Education
NOTE: This is the Professional Version. CONSUMERS: Click here for the Consumer Version

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