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POEMS Syndrome

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


Jennifer M. Barker

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

Reviewed/Revised Apr 2023
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POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) is a nonautoimmune polyglandular deficiency syndrome.

Symptoms and Signs of POEMS Syndrome

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 of POEMS Syndrome

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 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. Khouri J, Nakashima M, Wong S: Update on the diagnosis and treatment of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) syndrome. JAMA Oncol 7(9):1383-1391, 2021. doi: 10.1001/jamaoncol.2021.0586

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NOTE: This is the Professional Version. CONSUMERS: View Consumer Version
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