Hematopoietic cell transplantation utilization and outcomes for primary plasma cell leukemia in the current era

Multiple myeloma: EHA-ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up†

“…In a recent study, 71 patients (median age 56 years) with PPCL underwent an allo-SCT and the 4-year outcomes were: nonrelapse mortality 12%, PFS 19% and OS 31%. 83 Thus, in patients younger than 50 years of age with a suitable donor, a myeloablative allo-SCT can be considered. Otherwise, a tandem transplant with an ASCT followed by a reducedintensity conditioning allo-SCT if a related or an unrelated donor is available can be considered [IV, C].…”

First-line treatment and survival of newly diagnosed primary plasma cell leukemia patients in the Netherlands: a population-based study, 1989-2018

“…Also, the selection of comparatively fit patients fulfilling the strict inclusion criteria of trials-as compared to the inclusion of all pPCL patients identified in population-based registries-may result in differences between the studies. Concerning stem cell transplantation, the OS in transplant-eligible pPCL patients in three large retrospective series 6,13,14 was superior, as compared to the OS of pPCL patients aged ≤65 years diagnosed in 2014-2018 in our population-based study-of whom 63% received autoSCT. However, it is unknown which proportion of patients in the retrospective series 6,13,14 was planned to undergo autoSCT, but eventually did not receive this treatment because of either early progression or early death.…”

Management of Primary Plasma Cell Leukemia Remains Challenging Even in the Era of Novel Agents

“…In HSCT-ineligible patients, the optimal strategy is unknown but a several cycles of remission induction therapy, followed by long term multi-agent maintenance therapy containing PI and an immunomodulatory agent (IMiD) may provide durable remission. A recently published multicenter retrospective analysis of 348 patients with PCL by Dhakal et al 10 concluded that despite incorporation of modern induction therapy containing novel agents and despite increased utilization of both autologous and allogeneic HSCT in the last several years, the survival of PCL patients has not improved in comparison to the historical cohort of PCL patients prior to the widespread use of novel agents. The study underscored that the main reason for the lack of improvement in the survival despite utilization of modern therapies is due to the high relapse rate after HSCT (~76% relapsed at 4 years post-HSCT).…”

“…The study underscored that the main reason for the lack of improvement in the survival despite utilization of modern therapies is due to the high relapse rate after HSCT (~76% relapsed at 4 years post-HSCT). 10 Allogeneic HSCT is only suitable for a minority of PCL patients as shown in a few small studies 10 demonstrating initial good response, a small PFS benefit of 19% at 4 years and overall survival (OS) of only 31% at 4 years. Small studies of tandem double autologous or tandem autologous/allogeneic HSCT showed limited benefit as well.…”