Risk factors for and outcomes of patients with POEMS syndrome who experience progression after first-line treatment

Taxiarchis Kourelis, F. K. Buadi, Morie Gertz, Martha Lacy, Shaji K Kumar, Prashant Kapoor, R. S. Go, J. A. Lust, S. R. Hayman, S Vincent Rajkumar, S. R. Zeldenrust, Stephen J Russell, David M Dingli, Yi Lin, N. Leung, Y. L. Hwa, Wilson Gonsalves, R. A. Kyle, Angela Dispenzieri

Research output: Contribution to journalArticle

15 Citations (Scopus)

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 languageEnglish (US)
JournalLeukemia
DOIs
StateAccepted/In press - Dec 16 2015

Fingerprint

POEMS Syndrome
Recurrence
Therapeutics
Disease-Free Survival
Documentation
Vascular Endothelial Growth Factor A
Signs and Symptoms
Publications
Albumins
Leukemia
Multivariate Analysis
Skin

ASJC Scopus subject areas

  • Hematology
  • Cancer Research
  • Anesthesiology and Pain Medicine

Cite this

@article{a35a4fac7935465da72b3c799ab98ffd,
title = "Risk factors for and outcomes of patients with POEMS syndrome who experience progression after first-line treatment",
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.",
author = "Taxiarchis Kourelis and Buadi, {F. K.} and Morie Gertz and Martha Lacy and Kumar, {Shaji K} and Prashant Kapoor and Go, {R. S.} and Lust, {J. A.} and Hayman, {S. R.} and Rajkumar, {S Vincent} and Zeldenrust, {S. R.} and Russell, {Stephen J} and Dingli, {David M} and Yi Lin and N. Leung and Hwa, {Y. L.} and Wilson Gonsalves and Kyle, {R. A.} and Angela Dispenzieri",
year = "2015",
month = "12",
day = "16",
doi = "10.1038/leu.2015.344",
language = "English (US)",
journal = "Leukemia",
issn = "0887-6924",
publisher = "Nature Publishing Group",

}

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.

UR - http://www.scopus.com/inward/record.url?scp=84954350467&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84954350467&partnerID=8YFLogxK

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 -