POEMS Syndrome: Real World Experience in Diagnosis and Systemic Therapy - 108 Patients Multicenter Analysis

Artur Jurczyszyn, Jorge J. Castillo, Magdalena Olszewska-Szopa, Lalit Kumar, Santiago Thibaud, Joshua Richter, Kari Flicker, Mark Fiala, Ravi Vij, Shuhua Yi, Fang Xu, Rebecca Silbermann, Carmen Montes Gaisan, Enrique M. Ocio, Anna Waszczuk-Gajda, Edvan De Queiroz Crusoe, Aleksander Salomon-Perzyński, Iwona Hus, Julio Davila Valls, Alessandro GozzettiJacek Czepiel, Katarzyna Krzanowska, Aimee Chappell, S. K. Chellapuram, Anna Suska, David H. Vesole

Research output: Contribution to journalArticlepeer-review

Abstract

POEMS syndrome, a rare plasma cell disorder, is challenging both in the diagnostic and therapeutic management. We present real word retrospective analysis of 108 cases analyzing clinical features and therapeutic modes. We compare our results with the available literature. This is the first description with such wide use of proteasome inhibitors in first line treatment. POEMS (Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) syndrome is a rare and challenging plasma cell disorder, both in the diagnostic and therapeutic management of the disease. Currently, the literature on POEMS is sparse with most evidence being case reports and small case studies. We present a retrospective real world experience of 108 patients with POEMS. We analyzed the clinical features and therapeutic interventions. Regarding clinical features, our findings demonstrated that skin lesions, thrombocythemia and polycythemia were present less frequently than reported previously. Regarding clinical interventions, this is one of the largest analyses of front line treatment in POEMS and the first one to include frequent utilization of proteasome inhibitors (37%). Bortezomib monotherapy was the most effective therapy achieving complete remission/very good partial remissions (CR/VGPR) in 69% of patients. Thirty percent of patients proceeded to planned autologous stem cell transplant (ASCT) as part of the front-line treatment resulting in statistically superior progression-free (PFS) and overall survival (OS) compared to non-ASCT treated patients (P= .003). In multivariate analysis, anemia, thrombocytopenia, and as age over 60 were associated with a negative impact on patient outcomes.

Original languageEnglish (US)
JournalClinical Lymphoma, Myeloma and Leukemia
DOIs
StateAccepted/In press - 2021
Externally publishedYes

Keywords

  • POEMS
  • plasma cell dyscrsia
  • standard of care

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

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