Journal article
Outcomes of pPCL Diagnosed Using the IMWG 2021 Consensus Definition: A Retrospective Multicenter Analysis
Cancers, Vol.18(1), 177
01/05/2026
DOI: 10.3390/cancers18010177
PMCID: PMC12785085
PMID: 41514683
Abstract
Primary plasma cell leukemia (pPCL) represents the most aggressive plasma cell dyscrasia with a poor prognosis and survival of <3 years. The International Myeloma Working Group (IMWG) adopted more inclusive diagnostic criteria for pPCL in 2021, including patients with 5% or more circulating plasma cells (down from 20%). Most published studies of pPCL do not include patients who meet the criteria for pPCL based on the newer diagnostic guidelines, and the data on the optimal treatment of pPCL is scarce. In our multi-center retrospective analysis, we report data on treatment regimens used in 67 pPCL patients to characterize outcomes in this population.
We included patients with newly diagnosed pPCL between 2010 and 2023 based on the 2021 IMWG definition at one of three academic centers.
Our results suggest significant improvement in overall response rate (ORR) and progression-free survival (PFS) with the use of autologous stem cell transplant, but without additional benefit for a tandem transplant. The presence of high-risk cytogenetics was an independent risk factor for progression in the cohort.
Our dataset represents one of the largest cohorts to date using the expanded definition of pPCL adopted by the IMWG in 2021 and stresses the importance of taking pPCL patients to transplant. Unfortunately, our study was not powered to determine the efficacy of individual induction and maintenance regimens, and many patients diagnosed with pPCL are ineligible for transplant based on end-organ damage at diagnosis or from disease that is refractory to induction therapy, underscoring the need for early diagnosis and treatment in hopes of preserving transplant eligibility.
Details
- Title: Subtitle
- Outcomes of pPCL Diagnosed Using the IMWG 2021 Consensus Definition: A Retrospective Multicenter Analysis
- Creators
- Priyanka Venkatesh - University of Alabama at BirminghamRazan Mansour - Yale Cancer CenterYara Shatnawi - Jordan University of Science and TechnologyAkhil Jain - University of Iowa Hospitals and ClinicsChristopher Strouse - University of IowaNausheen Ahmed - University of Kansas Medical CenterMuhammad Umair Mushtaq - University of Kansas Medical CenterAl-Ola Abdallah - University of Kansas Medical CenterShebli Atrash - Levine Cancer InstituteBarry Paul - Levine Cancer Institute
- Resource Type
- Journal article
- Publication Details
- Cancers, Vol.18(1), 177
- DOI
- 10.3390/cancers18010177
- PMID
- 41514683
- PMCID
- PMC12785085
- NLM abbreviation
- Cancers (Basel)
- ISSN
- 2072-6694
- eISSN
- 2072-6694
- Publisher
- MDPI
- Grant note
- U.S. Myeloma Innovations and Research Collaborative (USMIRC)
We wish to thank the research and data staff at the participating institutions for their contributions to this work. The authors gratefully acknowledge the support of the U.S. Myeloma Innovations and Research Collaborative (USMIRC) for its contributions to this work, including assistance with study design, critical review of the manuscript, and administrative coordination.
- Language
- English
- Date published
- 01/05/2026
- Academic Unit
- Hematology, Oncology, and Blood & Marrow Transplantation; Internal Medicine
- Record Identifier
- 9985116169702771
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