POEMS syndrome
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Andrew J. Cowan, MD University of Washington Seattle, WA  andrewcowanmd |
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)
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Rd: Revlimid (Lenalidomide) & low-dose dexamethasone
Regimen
| Study | Evidence |
|---|---|
| Nozza et al. 2017 | Phase II, <20 pts |
Targeted therapy
- Lenalidomide (Revlimid) 25 mg PO once per day on days 1 to 21
- Dexamethasone (Decadron) 40 mg PO once per day on days 1, 8, 15, 22
Supportive medications
- 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
- 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