Clinical features and outcomes of plasma cell leukemia: a single-institution experience in the era of novel agents.
Bottom Line: OS was inferior to that of 313 consecutive patients with multiple myeloma (MM) treated in the same period, even when compared with a subset of 47 MM with high-risk cytogenetics.Although our data are limited by the small sample size, we conclude that novel agents may modestly improve survival in patients with PCL, when compared to historical controls.Novel therapies do not seem to overcome the negative prognosis of PCL as compared with MM.
Affiliation: Penn State Hershey Cancer Institute, Hershey;
Plasma cell leukemia (PCL) is a rare hematologic malignancy with aggressive clinical and biologic features. Data regarding its prognosis with the use of the novel agents, i.e., the immunomodulatory drugs thalidomide and lenalidomide, and the proteasome inhibitor bortezomib, are limited. We retrospectively reviewed clinical outcomes, response to therapy, and survival of 17 patients seen at the Penn State Hershey Cancer Institute since the availability of novel agents (2006-2011). Twelve patients had primary PCL (pPCL), and 5 secondary PCL (sPCL). PCL was associated with aggressive clinicobiological features, such as high-risk cytogenetics, elevated serum beta-2-microglobulin and lactate dehydrogenase, International Staging System stage III, and rapid relapse after therapy. With the use of thalidomide, lenalidomide, and bortezomib in 53%, 53%, and 88% patients, respectively, median overall survival (OS) was 18 months in the whole group (95% confidence interval, 11-21 months), and 21 and 4 months in pPCL and sPCL, respectively (P=0.015). OS was inferior to that of 313 consecutive patients with multiple myeloma (MM) treated in the same period, even when compared with a subset of 47 MM with high-risk cytogenetics. Although our data are limited by the small sample size, we conclude that novel agents may modestly improve survival in patients with PCL, when compared to historical controls. Novel therapies do not seem to overcome the negative prognosis of PCL as compared with MM.
No MeSH data available.
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Mentions: Treatment included thalidomide-based regimen (9 pts, 53%), lenalidomide-based regimen (9 pts, 53%), bortezomib-based regimen (15 pts, 88%), single autologous stem cell transplantation (SCT) (4 pts, 24%), tandem autologous SCT (4 pts, 24%), and allogeneic SCT (2 pts, 12%). Response rate (RR) to first-line therapy, defined as PR or better, was seen in 14 patients (82%), and nCR/CR was obtained in 4 patients (24%). Median progression-free survival after the initial induction therapy was 10 months (range, 2–63), and 1.2 months (range, 0.5–8.8) in pPCL and sPCL, respectively (P=0.0011). The median OS for all patients was 18 months (95% C.I., 11–21 months), and 21 and 4 months in pPCL and sPCL, respectively (p=0.0154). Median OS in patients with sPCL, when calculated from the initial diagnosis of MM, was 31 months (95% confidence interval, 28–46). Median OS in the whole group of PCL was not only inferior to that observed in 313 consecutive MM patients treated in the same period (91 months, P<0.01), but also inferior to the median OS in a subgroup of 47 consecutive MM patients with high-risk cytogenetics (44 months, P<0.01) (Figure 1A). OS was similar between pPCL and high-risk MM (Figure 1B). After a median follow-up of 25 months, 11 patients with PCL (65%) were dead, and the cause of death was progressive disease in 10 pts, and transplant-related mortality in 1 pt.
No MeSH data available.