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 Detalle_Publicacion

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

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.

 Fuente: Clin Lymphoma Myeloma Leuk . 2022 May;22(5):297-304

Editorial: Elsevier

 Año de publicación: 2022

Nº de páginas: 8

Tipo de publicación: Artículo de Revista

 DOI: 10.1016/j.clml.2021.10.007

ISSN: 2152-2650,2152-2669

Url de la publicación: ttps://doi.org/10.1016/j.clml.2021.10.007

Autores/as

JURCZYSZYN, ARTUR

CASTILLO, JORGE J

OLSZEWSKA-SZOPA, MAGDALENA

KUMAR, LALIT

THIBAUD, SANTIAGO

RICHTER, JOSHUA

FLICKER, KARI

FIALA, MARK

VIJ, RAVI

YI, SHUHUA

XU, FANG

SILBERMANN, REBECCA

GAISAN, CARMEN MONTES

WASZCZUK-GAJDA, ANNA

CRUSOE, EDVAN DE QUEIROZ

SALOMON-PERZYNSKI, ALEKSANDER

HUS, IWONA

VALLS, JULIO DAVILA

GOZZETTI, ALESSANDRO