ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (5)
POEMS syndrome
Other Resources UpToDate PubMed

POEMS syndrome

Contributors: Nneka I. Comfere MD, Jeffrey M. Cohen MD, Susan Burgin MD, Paritosh Prasad MD
Other Resources UpToDate PubMed

Synopsis

POEMS syndrome is a multisystem disorder associated with underlying plasma cell dyscrasia. POEMS is a syndrome of polyneuropathy, organomegaly, endocrinopathy, multiple myeloma, and skin changes and sclerotic bone changes.

While the etiology of POEMS syndrome is not known, it is attributed to chronic inflammation (cytokine overproduction) resulting in microangiopathy, edema, increased vascular permeability, and neovascularization. Polyneuropathy, pulmonary hypertension, leukocytosis, and thrombocytosis are possible consequences.

POEMS syndrome should be considered in patients with polyneuropathy and evidence of a monoclonal plasma cell disorder. It is a rare disorder and affects patients predominantly in their fifth and sixth decades of life, with a median age of 51 years and a slight male predominance. While all patients with POEMS syndrome will have polyneuropathy and monoclonal cell disorders, and the vast majority will have osteosclerotic bone lesions, only about two-thirds will have associated skin changes and endocrinopathy. About one-half will have concurrent organomegaly, and about one-third will have fatigue and weight loss. Castleman disease is seen in about 15% of cases.

Criteria used for diagnosis include the following.

Mandatory major criteria:
  • Polyneuropathy that is frequently demyelinating (100%)
  • Monoclonal plasma cell proliferation (100%) – usually immunoglobulin G (IgG), almost always lambda chain
Other major criteria include:
  • Castleman disease (angiofollicular lymph node hyperplasia)
  • Marked elevation of serum vascular endothelial growth factor (VEGF) levels
  • Extravascular volume overload (ascites, pleural effusions)
  • Endocrinopathy that may have affected several glands (67%-84%)
  • Bone lesions – usually sclerotic (27%-97%)
  • Multiple skin changes (detailed below) (68%-89%)
  • Papilledema (29%-64%)
  • Thrombocytosis (54%-88%)
For more information, see OMIM.

Codes

ICD10CM:
D47.9 – Neoplasm of uncertain behavior of lymphoid, hematopoietic and related tissue, unspecified

SNOMEDCT:
79268002 – POEMS syndrome

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Differential diagnosis of hyperpigmentation and sclerotic skin:
Differential diagnosis of sclerodermoid plaques:
See also the differential diagnoses of glomeruloid hemangioma and hypertrichosis.

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed:03/24/2021
Last Updated:03/24/2021
Copyright © 2021 VisualDx®. All rights reserved.
POEMS syndrome
POEMS syndrome (Systemic) : Fatigue, Hepatosplenomegaly, Ascites, Lymphadenopathy, Papilledema, Pleural effusion
Clinical image of POEMS syndrome
Copyright © 2021 VisualDx®. All rights reserved.