European Myeloma Network Group review and consensus statement on primary plasma cell leukemia
Language English Country England, Great Britain Media print-electronic
Document type Journal Article, Review
PubMed
39924085
DOI
10.1016/j.annonc.2025.01.022
PII: S0923-7534(25)00058-4
Knihovny.cz E-resources
- Keywords
- Delphi consensus, EMN, guidelines, high-risk multiple myeloma, practical recommendations, primary plasma cell leukemia,
- MeSH
- Consensus MeSH
- Humans MeSH
- Multiple Myeloma * therapy MeSH
- Leukemia, Plasma Cell * therapy diagnosis pathology MeSH
- Prognosis MeSH
- Antineoplastic Combined Chemotherapy Protocols therapeutic use MeSH
- Practice Guidelines as Topic MeSH
- Check Tag
- Humans MeSH
- Publication type
- Journal Article MeSH
- Review MeSH
- Geographicals
- Europe MeSH
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
Cancer Center Clínica Universidad de Navarra CIMA IDISNA CIBERONC Pamplona Spain
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
European Myeloma Network Turin Italy
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
Hospital Clínic de Barcelona IDIBAPS University of Barcelona Barcelona Spain
Immuno Hematology Unit St Louis APHP Paris France
Istinye University Ankara Liv Hospital Kavaklıdere Ankara Turkey
National and Kapodistrian University of Athens Department of Therapeutics Athens Greece
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
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