[en] [en] 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.
Disciplines :
Hematology
Author, co-author :
Musto, P; 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: pellegrino.musto@uniba.it
Engelhardt, M ; University of Freiburg Medical Center, Faculty of Freiburg, Freiburg, Germany
van de Donk, N W C J; Amsterdam UMC, Vrije Universiteit Amsterdam, Department of Hematology, Cancer Center Amsterdam, Amsterdam, the Netherlands
Gay, F; 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
Terpos, E ; Department of Clinical Therapeutics, National and Kapodistrian University of Athens, School of Medicine, Alexandra General Hospital, Athens, Greece
Einsele, H; University Hospital Würzburg, Department of Internal Medicine II, Würzburg, Germany
Fernández de Larrea, C ; Hospital Clínic de Barcelona, IDIBAPS, University of Barcelona, Barcelona, Spain
Sgherza, N; Hematology and Stem Cell Transplantation Unit, AOU Consorziale Policlinico, Bari, Italy
Bolli, N; Department of Oncology and Onco-Hematology, University of Milan, Milan, Italy, Section of Hematology, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
Katodritou, E; Department of Hematology, Theagenion Cancer Hospital, Thessaloniki, Greece
Gentile, M; Hematology Unit, Department of Onco-hematology, A.O. of Cosenza, Cosenza, Italy, Department of Pharmacy, Health and Nutritional Science, University of Calabria, Rende, Italy
Royer, B; Immuno-Hematology Unit, St Louis, APHP, Paris, France
Derudas, D ; Department of Hematology and Bone Marrow Transplant Center, Oncologic Hospital "A. Businco", Cagliari, Italy
Jelinek, T; Department of Hematooncology, University Hospital Ostrava and Faculty of Medicine, University of Ostrava, Ostrava, Czech Republic
Zamagni, E; IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seràgnoli", Bologna, Italy, Dipartimento di Scienze Mediche e Chirurgiche, Università di Bologna, Bologna, Italy
Rosiñol, L; Hematology Department, Amyloidosis and Myeloma Unit, Hospital Clínic, IDIBAPS, Barcelona, Spain
Paiva, B; Clínica Universidad de Navarra, Centro de Investigación Médica Aplicada, Universidad de Navarra, CCUN, IDISNA, CIBER-ONC CB16/12/00369, Pamplona, Spain
Caers, Jo ; Université de Liège - ULiège > Département des sciences cliniques > Hématologie
Kaiser, M ; The Institute of Cancer Research and The Royal Marsden Hospital, London, UK
Beksac, M; Istinye University - Ankara Liv Hospital, Kavaklıdere, Ankara, Turkey
Hájek, R; Department of Haematooncology, University Hospital Ostrava, Ostrava, Czech Republic, Faculty of Medicine, University of Ostrava, Ostrava, Czech Republic
Spencer, A; Alfred Health-Monash University, Melbourne, Australia
Ludwig, H; Wilhelminen Cancer Research Institute, c/o Department of Medicine I, Center for Oncology and Hematology, Clinic Ottakring, Vienna, Austria
Cavo, M; IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seràgnoli", Bologna, Italy, Dipartimento di Scienze Mediche e Chirurgiche, Università di Bologna, Bologna, Italy
Bladé, J; Department of Hematology, Hospital Clínic, IDIBAPS, University of Barcelona, Barcelona, Spain
Moreau, P; Hematology Department, University Hospital Hôtel-Dieu, Nantes, France
Mateos, M-V; University Hospital of Salamanca/IBSAL/CIC/CIBERONC, Salamanca
San-Miguel, J F; Cancer Center, Clínica Universidad de Navarra, CIMA, IDISNA, CIBERONC, Pamplona, Spain
Dimopoulos, M A; National and Kapodistrian University of Athens, Department of Therapeutics, Athens, Greece
Boccadoro, M; European Myeloma Network (EMN), Turin, Italy
Sonneveld, P; Department of Hematology, Erasmus MC Cancer Institute, Rotterdam, the Netherlands
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