Difference between revisions of "POEMS syndrome"

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!colspan="2" align="center" style="color:white; font-size:125%; background-color:#de2d26"|'''Section editor'''
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|<big>[[User:Andrewc072|Andrew J. Cowan, MD]]<br>University of Washington<br>Seattle, WA</big><br>[[File:Social-twitter-icon.png|frameless|upright=0.1]][https://twitter.com/andrewcowanmd andrewcowanmd]<br>[https://www.linkedin.com/in/andrew-cowan-63b3a130/ LinkedIn]
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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
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<big>'''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</big>
 
 
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=Guidelines=
 
=Guidelines=
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'''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==
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*'''2018:''' Gavriatopoulou et al. [https://www.nature.com/articles/s41375-018-0209-7 European myeloma network recommendations on diagnosis and management of patients with rare plasma cell dyscrasias] [https://www.ncbi.nlm.nih.gov/pubmed/30038381 PubMed]
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*'''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}}==
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==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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<div class="toccolours" style="background-color:#eeeeee">
 
===Regimen {{#subobject:be34d7|Variant=1}}===
 
===Regimen {{#subobject:be34d7|Variant=1}}===
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!style="width: 25%"|Study
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!style="width: 33%"|Study
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!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]]
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!style="width: 33%"|Dates of enrollment
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!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
 
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|[https://onlinelibrary.wiley.com/doi/10.1111/bjh.14966/full Nozza et al. 2017]
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|[https://doi.org/10.1111/bjh.14966 Nozza et al. 2017]
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|style="background-color:#ffffbe"|Phase II, <20 pts
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|2009-10 to 2014-10
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|style="background-color:#ffffbe"|Phase 2, fewer than 20 pts
 
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====Chemotherapy====
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====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
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====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====
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====Supportive medications====
 
 
*[[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
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*[[Trimethoprim/Sulfamethoxazole_(Bactrim_DS)|Cotrimoxazole]] 800 mg PO twice per day two days per week
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*[[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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</div></div>
 
===References===
 
===References===
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# 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://onlinelibrary.wiley.com/doi/10.1111/bjh.14966/full link to original article] '''contains verified protocol''' [https://www.ncbi.nlm.nih.gov/pubmed/29048107 PubMed]
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# 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
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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]]

Latest revision as of 12:24, 15 July 2024

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Section editor
Samuelrubinstein.jpg
 Samuel M. Rubinstein, MD
University of North Carolina
Chapel Hill, NC, USA
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POEMS: Peripheral neuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, and Skin changes

1 regimens on this page
1 variants on this page


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

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

Glucocorticoid therapy

Supportive therapy

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 dosing details in manuscript have been reviewed by our editors PubMed EudraCT 2008-003202-33
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