Abstract
Although clinical improvement is almost universal with therapy in patients with POEMS (an acronym for polyneuropathy, organomegaly, endocrinopathies, monoclonal protein and a variety of skin changes) syndrome, outcomes and management of patients who relapse or progress (R/P) after first-line treatment have not been described. We retrospectively identified 262 patients with POEMS syndrome treated at the Mayo Clinic from 1974 to 2014 and who had follow-up information. The 5-year progression-free survival (PFS) and overall survival (OS) was 58% and 78%, respectively. Median time to R/P was 42 months. Seventy-nine patients (30%) had an R/P, with 52 (19%) experiencing a symptomatic R/P. Eighteen patients relapsed with symptoms or signs that were not documented at diagnosis. Median times to vascular endothelial growth factor, hematologic, radiographic and clinical R/P were 35 months (range, 4–327 months), 72 months (range, 4–327 months), 51 months (range, 4–327 months) and 48 months (range, 6–311 months), respectively. On multivariate analyses, low albumin at diagnosis and failure to achieve a complete hematologic response to first-line therapy were independent risk factors for PFS. Thirty patients had documentation of a second R/P at a median of 26 months from diagnosis of the first R/P. An early R/P was a risk factor for death, but most patients with an R/P had salvageable disease. A majority of patients are still without R/P at 5 years from diagnosis.Leukemia advance online publication, 15 January 2016; doi:10.1038/leu.2015.344.
| Original language | English (US) |
|---|---|
| Journal | Leukemia |
| DOIs | |
| State | Accepted/In press - Dec 16 2015 |
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ASJC Scopus subject areas
- Hematology
- Cancer Research
- Anesthesiology and Pain Medicine
Cite this
Risk factors for and outcomes of patients with POEMS syndrome who experience progression after first-line treatment. / Kourelis, Taxiarchis; Buadi, F. K.; Gertz, Morie; Lacy, Martha; Kumar, Shaji K; Kapoor, Prashant; Go, R. S.; Lust, J. A.; Hayman, S. R.; Rajkumar, S Vincent; Zeldenrust, S. R.; Russell, Stephen J; Dingli, David M; Lin, Yi; Leung, N.; Hwa, Y. L.; Gonsalves, Wilson; Kyle, R. A.; Dispenzieri, Angela.
In: Leukemia, 16.12.2015.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Risk factors for and outcomes of patients with POEMS syndrome who experience progression after first-line treatment
AU - Kourelis, Taxiarchis
AU - Buadi, F. K.
AU - Gertz, Morie
AU - Lacy, Martha
AU - Kumar, Shaji K
AU - Kapoor, Prashant
AU - Go, R. S.
AU - Lust, J. A.
AU - Hayman, S. R.
AU - Rajkumar, S Vincent
AU - Zeldenrust, S. R.
AU - Russell, Stephen J
AU - Dingli, David M
AU - Lin, Yi
AU - Leung, N.
AU - Hwa, Y. L.
AU - Gonsalves, Wilson
AU - Kyle, R. A.
AU - Dispenzieri, Angela
PY - 2015/12/16
Y1 - 2015/12/16
N2 - Although clinical improvement is almost universal with therapy in patients with POEMS (an acronym for polyneuropathy, organomegaly, endocrinopathies, monoclonal protein and a variety of skin changes) syndrome, outcomes and management of patients who relapse or progress (R/P) after first-line treatment have not been described. We retrospectively identified 262 patients with POEMS syndrome treated at the Mayo Clinic from 1974 to 2014 and who had follow-up information. The 5-year progression-free survival (PFS) and overall survival (OS) was 58% and 78%, respectively. Median time to R/P was 42 months. Seventy-nine patients (30%) had an R/P, with 52 (19%) experiencing a symptomatic R/P. Eighteen patients relapsed with symptoms or signs that were not documented at diagnosis. Median times to vascular endothelial growth factor, hematologic, radiographic and clinical R/P were 35 months (range, 4–327 months), 72 months (range, 4–327 months), 51 months (range, 4–327 months) and 48 months (range, 6–311 months), respectively. On multivariate analyses, low albumin at diagnosis and failure to achieve a complete hematologic response to first-line therapy were independent risk factors for PFS. Thirty patients had documentation of a second R/P at a median of 26 months from diagnosis of the first R/P. An early R/P was a risk factor for death, but most patients with an R/P had salvageable disease. A majority of patients are still without R/P at 5 years from diagnosis.Leukemia advance online publication, 15 January 2016; doi:10.1038/leu.2015.344.
AB - Although clinical improvement is almost universal with therapy in patients with POEMS (an acronym for polyneuropathy, organomegaly, endocrinopathies, monoclonal protein and a variety of skin changes) syndrome, outcomes and management of patients who relapse or progress (R/P) after first-line treatment have not been described. We retrospectively identified 262 patients with POEMS syndrome treated at the Mayo Clinic from 1974 to 2014 and who had follow-up information. The 5-year progression-free survival (PFS) and overall survival (OS) was 58% and 78%, respectively. Median time to R/P was 42 months. Seventy-nine patients (30%) had an R/P, with 52 (19%) experiencing a symptomatic R/P. Eighteen patients relapsed with symptoms or signs that were not documented at diagnosis. Median times to vascular endothelial growth factor, hematologic, radiographic and clinical R/P were 35 months (range, 4–327 months), 72 months (range, 4–327 months), 51 months (range, 4–327 months) and 48 months (range, 6–311 months), respectively. On multivariate analyses, low albumin at diagnosis and failure to achieve a complete hematologic response to first-line therapy were independent risk factors for PFS. Thirty patients had documentation of a second R/P at a median of 26 months from diagnosis of the first R/P. An early R/P was a risk factor for death, but most patients with an R/P had salvageable disease. A majority of patients are still without R/P at 5 years from diagnosis.Leukemia advance online publication, 15 January 2016; doi:10.1038/leu.2015.344.
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U2 - 10.1038/leu.2015.344
DO - 10.1038/leu.2015.344
M3 - Article
C2 - 26669974
AN - SCOPUS:84954350467
JO - Leukemia
JF - Leukemia
SN - 0887-6924
ER -