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J Korean Med Sci.  2024 Jan;39(2):e5. 10.3346/jkms.2024.39.e5.

A Multicenter Analysis of Clinical Features and Long-Term Outcomes of POEMS Syndrome in Korea

Affiliations
  • 1Division of Hematology, Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
  • 2Department of Hematology, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 3Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
  • 4Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 5Department of Internal Medicine, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea
  • 6Department of Internal Medicine, Korea University Anam Hospital, Korea University College of Medicine, Seoul, Korea
  • 7Department of Internal Medicine, Gachon University Gil Medical Center, Gachon University College of Medicine, Incheon, Korea
  • 8Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea

Abstract

Background
POEMS syndrome is a rare form of plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal proteins, and skin changes. Owing to its low incidence, there are few reports regarding this syndrome. This multicenter study included 84 patients diagnosed with POEMS syndrome in South Korea.
Methods
We retrospectively evaluated 84 patients diagnosed with POEMS syndrome at 8 hospitals in South Korea between January 2000 and October 2022. The clinical characteristics and treatment outcomes were analyzed.
Results
The median patient age was 53 years (range, 26–77 years), and 63.1% of the patients were male. All patients had peripheral neuropathy, and 81 (96.4%) had monoclonal plasma cell proliferation. Plasma vascular endothelial growth factor levels were available for 32 patients with a median of 821 pg/mL (range, 26–12,900 pg/mL). Other common features included skin changes (54.2%), volume overload (71.4%), and organomegaly (72.6%). Of the 84 patients, 75 received initial treatment (local radiotherapy, 6 [8.0%]; chemotherapy, 17 [22.7%]; both chemotherapy and local radiotherapy, 9 [12.0%]), upfront autologous stem cell transplantation (ASCT), 43 (57.3%; with induction chemotherapy, n = 12, 16.0%; without induction chemotherapy, n = 31, 41.3%). The median follow-up duration was 40.7 months. The 5-year overall survival (OS) was 78%, and the 5-year progression-free survival (PFS) was 55%. Patients who underwent upfront ASCT and were diagnosed after 2014 had a longer OS and PFS.
Conclusion
The demographics of Korean patients with POEMS syndrome were similar to those reported previously. Because of the introduction of new treatment agents and the reduced rate of transplant-related mortality related to ASCT, the treatment outcomes of Korean patients with POEMS syndrome have improved in recent years.

Keyword

POEMS Syndrome; Long-Term Outcome; Autologous Stem Cell Transplantation

Figure

  • Fig. 1 OS (A) and PFS (B) of all treated patients (n = 75).OS = overall survival, PFS = progression-free survival.

  • Fig. 2 Overall survival (A) and progression-free survival (B) of patients who underwent autologous stem cell transplantation, stratified by eGFR (cutoff: 30 mL/min/1.73 m2; n = 43).eGFR = estimated glomerular filtration rate.

  • Fig. 3 Overall survival (A) and progression-free survival (B) of high-risk group patients by ASCT (n = 31).ASCT = autologous stem cell transplantation.


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