Plasma Cell Leukemia: A Multicenter Retrospective Study of 130 Patients

Chineke, Iloabueke; Wertheim, Betsy C.; Roe, Denise J.; Larsen, Ashley; Vardell, Victoria A.; Sborov, Douglas W.; Green, Damian J.; Liedtke, Michaela; Okoniewski, Marie; Wazir, Mohammed; Nadeem, Omar; Schachter, Levanto; Coffey, David G.; Gowin, Krisstina; DeGraaff, Dominique

doi:10.1182/blood-2022-164713

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“…This is probably secondary to the selection bias rather than the systemic assessment of the regimen and is likely explained by the introduction of DVT-PACE in a refractory setting after the failure of upfront induction, given the chemo-refractory nature of this population and with deletion of 17p detected in more than half of the patients. The OS in this study is similar to what has been reported from a recent multicenter retrospective study of 150 patients with a median OS 12.6 months [29].…”

Section: Discussionsupporting

confidence: 90%

“…PCL is a highly proliferative stage in MM [13]. The prognosis is poor and survival varies from a few years in newly diagnosed pPCL to a few months in sPCL [14,15]. The clinical and biological features also vary; pPCL is associated with a lower age at diagnosis, extramedullary disease (EMD), higher creatinine and beta-2 microglobulin (B2M), hypodiploidy, t (11;14), and longer OS in comparison to sPCL, which tends to present with a higher age at diagnosis, bony involvement, lower platelet count, high serum M-protein, hyperdiploidy, and much shorter OS [16].…”

Section: Introductionmentioning

confidence: 99%

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A Clinical Perspective on Plasma Cell Leukemia: A Single-Center Experience

Li,

Kamangar,

Holtzman

et al. 2024

Cancers

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Circulating plasma cells (CPCs) are detected in most multiple myeloma (MM) patients, both at diagnosis and on relapse. A small subset, plasma cell leukemia (PCL), represents a different biology and has a poor prognosis. In this retrospective analysis, we evaluated patients with primary (pPCL, n = 35) or secondary (sPCL, n = 49), with ≥5% CPCs and a smaller subset with lower CPCs of 1–4% (n = 20). The median age was 61 years; 45% were men and 54% were Black. High-risk cytogenetics were found in 87% and extramedullary disease in 47%. For the entire cohort, 75% received a proteasome inhibitor, 70% chemotherapy, 54% an immunomodulatory drug, 24% a daratumumab-based regimen and 26% an autologous stem cell transplant (ASCT). The treatments marginally improved the overall survival (OS) for pPCL vs. sPCL (13 vs. 3.5 months p = 0.002). However, the 5-year survival for the whole cohort was dismal at 11%. High-risk cytogenetics, low platelets, extramedullary disease and high LDH were independently associated with poor outcomes. Further research is urgently needed to expand the treatment options and improve the outcomes in PCL.

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Section: Discussionsupporting

confidence: 90%