The role of frontline autologous stem cell transplantation for primary plasma cell leukemia: a retrospective multicenter study (KMM160).

Sung Hoon Jung ,Cheolwon Suh,Ho Sup Lee,Jae Hoon Lee,Jin Seok Kim,Dok Hyun Yoon,Seong Kyu Park,Min Kyoung Kim,Chul Won Choi,Hyo Jung Kim,Sang Kyun Sohn,Kihyun Kım ,Yeung Chul Mun ,Ho Young Yhim ,Je Jung Lee ,Soo Mee Bang ,Chang Ki Min ,Sung Soo Yoon ,Jae Cheol Jo ,Ho Jin Shin

doi:10.18632/oncotarget.18535

Abstract

Primary plasma cell leukemia (pPCL) is a rare and aggressive plasma cell neoplasm, with rapidly progressing clinical course. We evaluated the treatment status and survival outcomes of 69 Korean patients with pPCL. Of them, 59 patients were treated; 15 (25.4%) were treated initially with novel agent-based regimens with upfront autologous stem cell transplantation (ASCT), 7 (11.9%) with conventional chemotherapy with upfront ASCT, 21 (35.6%) with novel agent-based regimens only, and 16 (27.1%) were treated with conventional chemotherapy alone. Overall response rates after initial therapy were significantly higher in patients treated with novel agent-based regimens compared with those treated with conventional chemotherapies (75% vs. 43.4%, P = 0.026). Median progression-free survival (PFS) and overall survival (OS) were 12.2 months and 16.1 months, respectively. The median PFS of the four treatment groups–conventional chemotherapy alone, novel agents alone, conventional chemotherapy with ASCT, and novel agents with ASCT–were 1.2, 9.0, 10.5, and 26.4 months, respectively (P < 0.001); the median OS of the four treatment groups were 2.9, 12.3, 14.1, and 31.1 months, respectively (P < 0.001). The median OS was also significantly better in the patients with novel agents with ASCT versus other patients. In a multivariate analysis, an increased lactate dehydrogenase level, low albumin (< 3.5 g/dL), and non-CR after front-line treatment were independently associated with poor PFS and OS. In conclusion, the use of novel agent-based therapy with ASCT and achieving a deep response to front-line treatment are important in expecting improved PFS and OS in patients with pPCL.

Highlights

Plasma cell leukemia (PCL) is a rare and highly aggressive plasma cell dyscrasia that occurs in 1–4% of patients with multiple myeloma (MM) [1, 2]
Over the last two decades, the development of more effective agents and increased use of autologous stem cell transplantation (ASCT) have resulted in improvements in survival outcome in patients with MM
A few retrospective studies have shown that treatment with novel agents induced higher response rates and prolonged survival than conventional chemotherapies

Summary

Introduction

Plasma cell leukemia (PCL) is a rare and highly aggressive plasma cell dyscrasia that occurs in 1–4% of patients with multiple myeloma (MM) [1, 2]. It is defined by the presence of more than 20% plasma cells in peripheral blood and an absolute plasma cell count ≥ 2 × 109/L [3, 4]. In a study investigating gene expression profiles in 21 patients with pPCL, a 503gene signature, especially related to the NFκB pathway, structural organization of the cell, and cell adhesion/ migration differed from that in MM [12]

Methods

Results

Conclusion