POEMS syndrome

Section editor
	Andrew J. Cowan, MD University of Washington Seattle, WA LinkedIn

POEMS: Peripheral neuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, and Skin changes

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Guidelines

EMN

• 2018: Gavriatopoulou et al. European myeloma network recommendations on diagnosis and management of patients with rare plasma cell dyscrasias PubMed

All lines of therapy

RD

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RD: Revlimid (Lenalidomide) & Dexamethasone

Regimen

Study	Evidence
Nozza et al. 2017	Phase II, <20 pts

Chemotherapy

• Lenalidomide (Revlimid) 25 mg PO once per day on days 1 to 21
• Dexamethasone (Decadron) 40 mg PO once per week

Supportive medications

• Aspirin 100 mg PO once per day
  • LMWH in "intolerant" patients
• Cotrimoxazole 800 mg PO twice per day two days per week

Monthly cycles, continued until progression or excess toxicity

References

1. Nozza A, Terenghi F, Gallia F, Adami F, Briani C, Merlini G, Giordano L, Santoro A, Nobile-Orazio E. Lenalidomide and dexamethasone in patients with POEMS syndrome: results of a prospective, open-label trial. Br J Haematol. 2017 Dec;179(5):748-755. Epub 2017 Oct 19. link to original article contains verified protocol PubMed