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

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机构： [1]Plasma Cell Dyscrasia Center, Department of Hematology Faculty of Medicine, Jagiellonian University Medical College, Cracow, Poland [2]Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA [3]Department of Hematology, Blood Neoplasms and Bone Marrow Transplantation, Wroclaw Medical University, Wroclaw, Poland [4]Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India [5]Mount Sinai Medical Center, New York, NY [6]Weill Cornell Medical College, New York, NY [7]Division of Oncology, Section of Bone Marrow Transplant & Leukemia, Washington University School of Medicine, Saint Louis, MO [8]State Key Laboratory of Experimental Hematology National Clinical Research Center for Blood Diseases Institute of Hematology and Blood Disease Hospital Chinese, Academy of Medical Sciences and Peking Union Medical College Tianjin China [9]Department of Hematology, Mianyang Central Hospital, Mianyang, Sichuan, People’s Republic of China [10]Division of Hematology and Medical Oncology, Oregon Health and Sciences University, Knight Cancer Institute, Portland [11]University Hospital Marques de Valdecilla (IDIVAL), University of Cantabria, Santander, Spain [12]Department of Hematology, Transplantation and Internal Medicine, Medical University of Warsaw, Poland [13]Universidade Federal da Bahia (UFBA), Hospital Universitário Professor Edgard Santos, Salvador, BA, Brazil [14]Department of Hematology, Institute of Hematology and Transfusion Medicine, Warsaw, Poland [15]Complejo Asistencial de Avila, Avila, Spain [16]Division of Hematology, Department of Medical Science, Surgery and Neuroscience, University of Siena, Siena, Italy [17]Department of Infectious and Tropical Diseases, Jagiellonian University Medical College, Cracow, Poland [18]Department of Nephrology, Jagiellonian University Medical College, Kraków, Poland [19]Department of Hematology/Oncology, Georgetown University Hospital, Washington, DC [20]John Theurer Cancer Center at Hackensack Meridian School of Medicine, Hackensack, NJ

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关键词： POEMS standard of care plasma cell dyscrsia

摘要：

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.Copyright © 2021 The Author(s). Published by Elsevier Inc. All rights reserved.

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出版当年[2021]版：

大类 | 4 区医学

小类 | 4 区肿瘤学 4 区血液学

最新[2023]版：

大类 | 4 区医学

小类 | 4 区血液学 4 区肿瘤学

第一作者：

第一作者机构： [1]Plasma Cell Dyscrasia Center, Department of Hematology Faculty of Medicine, Jagiellonian University Medical College, Cracow, Poland [*1]Department of Hematology, Jagiellonian University Medical College, 17 Kopernika Str. 31-000, Cracow, Poland.

通讯作者：

通讯机构： [1]Plasma Cell Dyscrasia Center, Department of Hematology Faculty of Medicine, Jagiellonian University Medical College, Cracow, Poland [*1]Department of Hematology, Jagiellonian University Medical College, 17 Kopernika Str. 31-000, Cracow, Poland.

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

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