POEMS syndrome

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

Lenalidomide & Dexamethasone (Rd)

Rd: Revlimid (Lenalidomide) & low-dose dexamethasone

Regimen

Study	Evidence
Nozza et al. 2017	Phase 2, fewer than 20 pts

Targeted therapy

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

Glucocorticoid therapy

• Dexamethasone (Decadron) 40 mg PO once per day on days 1, 8, 15, 22

Supportive therapy

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

28-day cycle for at least 6 cycles

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 dosing details in manuscript PubMed