Difference between revisions of "POEMS syndrome"
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==EMN== | ==EMN== | ||
*'''2018:''' Gavriatopoulou et al. [https://doi.org/10.1038/s41375-018-0209-7 European myeloma network recommendations on diagnosis and management of patients with rare plasma cell dyscrasias] [https://pubmed.ncbi.nlm.nih.gov/30038381 PubMed] | *'''2018:''' Gavriatopoulou et al. [https://doi.org/10.1038/s41375-018-0209-7 European myeloma network recommendations on diagnosis and management of patients with rare plasma cell dyscrasias] [https://pubmed.ncbi.nlm.nih.gov/30038381 PubMed] | ||
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=All lines of therapy= | =All lines of therapy= | ||
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==Lenalidomide & Dexamethasone (Rd) {{#subobject:dc6eae|Regimen=1}}== | ==Lenalidomide & Dexamethasone (Rd) {{#subobject:dc6eae|Regimen=1}}== | ||
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Rd: '''<u>R</u>'''evlimid (Lenalidomide) & low-dose '''<u>d</u>'''examethasone | Rd: '''<u>R</u>'''evlimid (Lenalidomide) & low-dose '''<u>d</u>'''examethasone | ||
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===Regimen {{#subobject:be34d7|Variant=1}}=== | ===Regimen {{#subobject:be34d7|Variant=1}}=== | ||
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====Targeted therapy==== | ====Targeted therapy==== | ||
*[[Lenalidomide (Revlimid)]] 25 mg PO once per day on days 1 to 21 | *[[Lenalidomide (Revlimid)]] 25 mg PO once per day on days 1 to 21 | ||
====Glucocorticoid therapy==== | ====Glucocorticoid therapy==== | ||
*[[Dexamethasone (Decadron)]] 40 mg PO once per day on days 1, 8, 15, 22 | *[[Dexamethasone (Decadron)]] 40 mg PO once per day on days 1, 8, 15, 22 | ||
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====Supportive therapy==== | ====Supportive therapy==== | ||
*[[Aspirin]] 100 mg PO once per day | *[[Aspirin]] 100 mg PO once per day | ||
**[[:Category:Low_molecular_weight_heparins|LMWH]] in "intolerant" patients | **[[:Category:Low_molecular_weight_heparins|LMWH]] in "intolerant" patients | ||
*[[Trimethoprim-Sulfamethoxazole_(Bactrim_DS)|Cotrimoxazole]] 800 mg PO twice per day two days per week | *[[Trimethoprim-Sulfamethoxazole_(Bactrim_DS)|Cotrimoxazole]] 800 mg PO twice per day two days per week | ||
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'''28-day cycle for at least 6 cycles''' | '''28-day cycle for at least 6 cycles''' | ||
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===References=== | ===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. [https://doi.org/10.1111/bjh.14966 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/29048107 PubMed] | # 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. [https://doi.org/10.1111/bjh.14966 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/29048107 PubMed] | ||
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[[Category:POEMS syndrome regimens]] | [[Category:POEMS syndrome regimens]] | ||
[[Category:Disease-specific pages]] | [[Category:Disease-specific pages]] | ||
[[Category:Plasma cell dyscrasias]] | [[Category:Plasma cell dyscrasias]] | ||
Revision as of 17:29, 28 February 2023
| Section editor | |
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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
1 regimens on this page
1 variants on this page
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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, <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
- 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