Difference between revisions of "POEMS syndrome"
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'''POEMS:''' '''<u>P</u>'''eripheral neuropathy, '''<u>O</u>'''rganomegaly, '''<u>E</u>'''ndocrinopathy, '''<u>M</u>'''onoclonal plasma cell disorder, and '''<u>S</u>'''kin changes | '''POEMS:''' '''<u>P</u>'''eripheral neuropathy, '''<u>O</u>'''rganomegaly, '''<u>E</u>'''ndocrinopathy, '''<u>M</u>'''onoclonal plasma cell disorder, and '''<u>S</u>'''kin changes | ||
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=Guidelines= | =Guidelines= | ||
+ | '''Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.''' | ||
==EMN== | ==EMN== | ||
− | *'''2018:''' Gavriatopoulou et al. [https:// | + | *'''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= | ||
− | + | ==Lenalidomide & Dexamethasone (Rd) {{#subobject:dc6eae|Regimen=1}}== | |
− | == | + | 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}}=== | ||
− | {| class="wikitable" style="width: | + | {| class="wikitable sortable" style="width: 60%; text-align:center;" |
− | !style="width: | + | !style="width: 33%"|Study |
− | !style="width: | + | !style="width: 33%"|Dates of enrollment |
+ | !style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
− | |[https:// | + | |[https://doi.org/10.1111/bjh.14966 Nozza et al. 2017] |
− | |style="background-color:#ffffbe"|Phase | + | |2009-10 to 2014-10 |
+ | |style="background-color:#ffffbe"|Phase 2, fewer than 20 pts | ||
|- | |- | ||
|} | |} | ||
− | ==== | + | <div class="toccolours" style="background-color:#b3e2cd"> |
+ | ====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 | ||
− | *[[Dexamethasone (Decadron)]] 40 mg PO once per | + | ====Glucocorticoid therapy==== |
− | + | *[[Dexamethasone (Decadron)]] 40 mg PO once per day on days 1, 8, 15, 22 | |
− | ====Supportive | + | ====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 | + | *[[Trimethoprim-Sulfamethoxazole_(Bactrim_DS)|Cotrimoxazole]] 800 mg PO twice per day two days per week |
− | + | '''28-day cycle for at least 6 cycles''' | |
− | ''' | + | </div></div> |
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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:// | + | # 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] '''dosing details in manuscript have been reviewed by our editors''' [https://pubmed.ncbi.nlm.nih.gov/29048107/ PubMed] EudraCT 2008-003202-33 |
− | + | [[Category:POEMS syndrome regimens]] | |
− | [[Category:POEMS regimens]] | ||
[[Category:Disease-specific pages]] | [[Category:Disease-specific pages]] | ||
[[Category:Plasma cell dyscrasias]] | [[Category:Plasma cell dyscrasias]] |
Latest revision as of 12:24, 15 July 2024
Section editor | |
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Samuel M. Rubinstein, MD University of North Carolina Chapel Hill, NC, USA |
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
Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.
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 | Dates of enrollment | Evidence |
---|---|---|
Nozza et al. 2017 | 2009-10 to 2014-10 | 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
- 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 dosing details in manuscript have been reviewed by our editors PubMed EudraCT 2008-003202-33