Abstract
Objective
To evaluate the clinical outcomes of patients with primary plasma cell leukemia (pPCL)
defined by 5% or greater clonal circulating plasma cells on peripheral blood smear
and treated with novel agent induction therapies.
Patients and Methods
A cohort of 68 patients with pPCL diagnosed at the Mayo Clinic in Rochester, Minnesota,
from January 1, 2000, to December 31, 2019, and treated with novel agent induction
therapies was evaluated.
Results
The median follow-up was 46 (95% CI, 41 to 90) months. The median bone marrow plasma
cell content was 85% (range, 10% to 100%) and median clonal circulaitng plasma cell
percentage on the peripheral blood smear was 26% (range, 5% to 93%). There was a preponderance
of t(11;14) primary cytogenetic abnormality in this cohort. The median time to next
therapy (TTNT) and overall survival (OS) for all patients with pPCL patients in this
cohort was 13 (95% CI, 9 to 17) and 23 (95% CI, 19 to 38) months, respectively. However,
when stratified by cytogenetic risk, the median TTNT and OS were 16 and 51 months
for standard risk vs 9 and 19 months for high risk (P=.01 for OS).
Conclusion
Primary plasma cell leukemia remains an aggressive disease with poor prognosis despite
novel agent–based therapies. Some patients have better than expected survival and
this phenomenon may be influenced by the absence of high-risk cytogenetics. Newer
treatment regimens are needed to improve the prognosis of this devastating disease.
Abbreviations and Acronyms:
allo-SCT (allogeneic stem cell transplantation), ASCT (autologous stem cell transplantation), CR (complete response), cPC (clonal plasma cell), FISH (fluorescence in situ hybridization), HR (hazard ratio), IMiD (immunomodulator), IMWG (International Myeloma Working Group), LDH (lactate dehydrogenase), LFU (lost to follow-up), MM (multiple myeloma), OS (overall survival), PB (peripheral blood), PCLI (plasma cell labeling index), PD (progressive disease), PI (proteasome inhibitor), pPCL (primary plasma cell leukemia), PR (partial response), SCT (stem cell transplantation), SD (stable disease), STD (standard), TTNT (time to next therapy), VDT-PACE (bortezomib, dexamethasone, thalidomide, cisplatin, doxorubicin, cyclophosphamide, and etoposide), VGPR (very good partial response)To read this article in full you will need to make a payment
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Article info
Footnotes
Grant Support: Research reported in this publication was supported by grants from the National Cancer Institute of the National Institutes of Health under award number K23CA218742. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. This research is also supported in part by the Marion Schwartz Foundation for Multiple Myeloma.
Potential Competing Interests: The authors declare no competing interests.
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