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European Myeloma Network Group review and consensus statement on primary plasma cell leukemia

P. Musto, M. Engelhardt, NWCJ. van de Donk, F. Gay, E. Terpos, H. Einsele, C. Fernández de Larrea, N. Sgherza, N. Bolli, E. Katodritou, M. Gentile, B. Royer, D. Derudas, T. Jelinek, E. Zamagni, L. Rosiñol, B. Paiva, J. Caers, M. Kaiser, M....

. 2025 ; 36 (4) : 361-374. [pub] 20250207

Jazyk angličtina Země Anglie, Velká Británie

Typ dokumentu časopisecké články, přehledy

Perzistentní odkaz   https://www.medvik.cz/link/bmc25009350

BACKGROUND: Primary plasma cell leukemia (PPCL) is the most aggressive disorder among plasma cell malignancies, with new diagnostic criteria recently established by the International Myeloma Working Group. Studies have shown that PPCL patients receiving a combination of novel agents, but not eligible for transplantation, may have a median survival up to 2 years, extended to 3 years or more in those undergoing transplant procedures. These findings remain unsatisfactory, particularly if compared with progresses obtained in multiple myeloma. DESIGN: A European Myeloma Network (EMN) expert panel reviewed the most recent literature and selected the areas of major concern in the management of PPCL by generating and rank ordering key questions using the criterion of clinical relevance. Multistep procedures were utilized to achieve a consensus on recommendations. The Delphi questionnaire method was used and a consensus of at least 80% was reached for all final statements. RESULTS: An extended overview of current biological, clinical, prognostic, and therapeutic aspects of PPCL, including ongoing and close to start clinical trials, is presented. Furthermore, updated guidelines for the management of PPCL and practical recommendations are provided, in the context of current knowledge about this disease, also looking at possible future perspectives to ameliorate the outcome of these patients. CONCLUSIONS: PPCL still remains an unmet clinical need. Notwithstanding, some not negligible progresses have been recently achieved. The European Myeloma Network panel strongly support ongoing and planned clinical trials, as well as biological studies based on novel technologies, strategies, and treatment options that could represent breakthroughs we have been waiting for too long.

Alfred Health Monash University Melbourne Australia

Amsterdam UMC Vrije Universiteit Amsterdam Department of Hematology Cancer Center Amsterdam Amsterdam the Netherlands

Cancer Center Clínica Universidad de Navarra CIMA IDISNA CIBERONC Pamplona Spain

Clínica Universidad de Navarra Centro de Investigación Médica Aplicada Universidad de Navarra CCUN IDISNA CIBER ONC CB16 12 00369 Pamplona Spain

Department of Clinical Therapeutics National and Kapodistrian University of Athens School of Medicine Alexandra General Hospital Athens Greece

Department of Haematooncology University Hospital Ostrava Ostrava Czech Republic

Department of Hematology and Bone Marrow Transplant Center Oncologic Hospital A Businco Cagliari Italy

Department of Hematology CHU de Liège Liège Belgium

Department of Hematology Erasmus MC Cancer Institute Rotterdam the Netherlands

Department of Hematology Hospital Clínic IDIBAPS University of Barcelona Barcelona Spain

Department of Hematology Theagenion Cancer Hospital Thessaloniki Greece

Department of Hematooncology University Hospital Ostrava and Faculty of Medicine University of Ostrava Ostrava Czech Republic

Department of Molecular Biotechnology and Health Sciences University of Torino Turin Italy

Department of Oncology and Onco Hematology University of Milan Milan Italy

Department of Pharmacy Health and Nutritional Science University of Calabria Rende Italy

Department of Precision and Regenerative Medicine and Ionian Area Aldo Moro University School of Medicine Bari Italy

Dipartimento di Scienze Mediche e Chirurgiche Università di Bologna Bologna Italy

Division of Hematology AOU Città della Salute e della Scienza di Torino University of Torino Turin Italy

European Myeloma Network Turin Italy

Faculty of Medicine University of Ostrava Ostrava Czech Republic

Hematology and Stem Cell Transplantation Unit AOU Consorziale Policlinico Bari Italy

Hematology Department Amyloidosis and Myeloma Unit Hospital Clínic IDIBAPS Barcelona Spain

Hematology Department University Hospital Hôtel Dieu Nantes France

Hematology Unit Department of Onco hematology A O of Cosenza Cosenza Italy

Hospital Clínic de Barcelona IDIBAPS University of Barcelona Barcelona Spain

Immuno Hematology Unit St Louis APHP Paris France

IRCCS Azienda Ospedaliero Universitaria di Bologna Istituto di Ematologia Seràgnoli Bologna Italy

Istinye University Ankara Liv Hospital Kavaklıdere Ankara Turkey

National and Kapodistrian University of Athens Department of Therapeutics Athens Greece

Section of Hematology Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milan Italy

The Institute of Cancer Research and The Royal Marsden Hospital London UK

University Hospital of Salamanca IBSAL CIC CIBERONC Salamanca

University Hospital Würzburg Department of Internal Medicine 2 Würzburg Germany

University of Freiburg Medical Center Faculty of Freiburg Freiburg Germany

Wilhelminen Cancer Research Institute c o Department of Medicine 1 Center for Oncology and Hematology Clinic Ottakring Vienna Austria

Citace poskytuje Crossref.org

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