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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 Apr 2021| Content last modified Apr 2021
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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 Overview of Plasma Cell Disorders Plasma cell disorders are a diverse group of disorders of unknown etiology characterized by Disproportionate proliferation of a single clone of B cells Presence of a structurally and electrophoretically... read more . Circulating cytokines (interleukin 1-beta, interleukin 6), vascular endothelial growth factor (VEGF), and tumor necrosis factor-alpha are also increased.

Symptoms and Signs

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).

Diagnosis

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 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 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

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