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Clinical Characteristics and Outcomes of Patients With Primary Plasma Cell Leukemia in the Era of Novel Agent Therapy



      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.


      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).


      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)
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