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

C Fernández de Larrea, J F San-Miguel, M-V Mateos, P Moreau, M Cavo, M Beksac, J Caers, B Paiva, L Rosiñol, E Zamagni, T Jelinek, D Derudas, B Royer, E Katodritou, N Bolli, N Sgherza, R Hájek, M Engelhardt, N W C J van de Donk, F Gay, H Ludwig, E Terpos, H Einsele, P Musto, J Bladé, M A Dimopoulos, M Gentile, P Sonneveld, A Spencer, M Kaiser, M Boccadoro

Journal: Annals of oncology : official journal of the European Society for Medical Oncology 2025;36(4):361-374

PMID: 39924085

Free Full Text on Elsevier Science View on PubMed

Abstract

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.

Address: Department of Precision and Regenerative Medicine and Ionian Area, "Aldo Moro" University School of Medicine, Bari, Italy; Hematology and Stem Cell Transplantation Unit, AOU Consorziale Policlinico, Bari, Italy. Electronic address: [email protected].; University of Freiburg Medical Center, Faculty of Freiburg, Freiburg, Germany.; Amsterdam UMC, Vrije Universiteit Amsterdam, Department of Hematology, Cancer Center Amsterdam, Amsterdam, the Netherlands.; Division of Hematology, AOU Città della Salute e della Scienza di Torino, University of Torino, Turin, Italy; Department of Molecular Biotechnology and Health Sciences, University of Torino, Turin, Italy.; Department of Clinical Therapeutics, National and Kapodistrian University of Athens, School of Medicine, Alexandra General Hospital, Athens, Greece.; University Hospital Würzburg, Department of Internal Medicine II, Würzburg, Germany.; Hospital Clínic de Barcelona, IDIBAPS, University of Barcelona, Barcelona, Spain.; Hematology and Stem Cell Transplantation Unit, AOU Consorziale Policlinico, Bari, Italy.; Department of Oncology and Onco-Hematology, University of Milan, Milan, Italy; Section of Hematology, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.; Department of Hematology, Theagenion Cancer Hospital, Thessaloniki, Greece.; Hematology Unit, Department of Onco-hematology, A.O. of Cosenza, Cosenza, Italy; Department of Pharmacy, Health and Nutritional Science, University of Calabria, Rende, Italy.; Immuno-Hematology Unit, St Louis, APHP, Paris, France.; Department of Hematology and Bone Marrow Transplant Center, Oncologic Hospital "A. Businco", Cagliari, Italy.; Department of Hematooncology, University Hospital Ostrava and Faculty of Medicine, University of Ostrava, Ostrava, Czech Republic.; IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seràgnoli", Bologna, Italy; Dipartimento di Scienze Mediche e Chirurgiche, Università di Bologna, Bologna, Italy.; Hematology Department, Amyloidosis and Myeloma Unit, Hospital Clínic, IDIBAPS, Barcelona, Spain.; Clínica Universidad de Navarra, Centro de Investigación Médica Aplicada, Universidad de Navarra, CCUN, IDISNA, CIBER-ONC CB16/12/00369, Pamplona, Spain.; Department of Hematology, CHU de Liège, Liège, Belgium.; The Institute of Cancer Research and The Royal Marsden Hospital, London, UK.; Istinye University - Ankara Liv Hospital, Kavaklıdere, Ankara, Turkey.; Department of Haematooncology, University Hospital Ostrava, Ostrava, Czech Republic; Faculty of Medicine, University of Ostrava, Ostrava, Czech Republic.; Alfred Health-Monash University, Melbourne, Australia.; Wilhelminen Cancer Research Institute, c/o Department of Medicine I, Center for Oncology and Hematology, Clinic Ottakring, Vienna, Austria.; Department of Hematology, Hospital Clínic, IDIBAPS, University of Barcelona, Barcelona, Spain.; Hematology Department, University Hospital Hôtel-Dieu, Nantes, France.; University Hospital of Salamanca/IBSAL/CIC/CIBERONC, Salamanca.; Cancer Center, Clínica Universidad de Navarra, CIMA, IDISNA, CIBERONC, Pamplona, Spain.; National and Kapodistrian University of Athens, Department of Therapeutics, Athens, Greece.; European Myeloma Network (EMN), Turin, Italy.; Department of Hematology, Erasmus MC Cancer Institute, Rotterdam, the Netherlands.

DOI: 10.1016/j.annonc.2025.01.022

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https://medlineplus.gov/multiplemyeloma.html

MeSH Terms: Humans, Leukemia, Plasma Cell, Consensus, Europe, Prognosis, Multiple Myeloma, Practice Guidelines as Topic, Antineoplastic Combined Chemotherapy Protocols