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Retrospective analysis of primary plasma cell leukemia in Kansai Myeloma Forum registry.

ABSTRACT: We retrospectively analyzed twenty-six patients with primary plasma cell leukemia (pPCL) registered from May 2005 until April 2015 by the Kansai Myeloma Forum. Twenty patients received novel agents (bortezomib or lenalidomide), and their median survival of was 34 months. The median survival of patients who underwent autologous stem cell transplantation (SCT) was 40 months, those undergoing allogeneic SCT 55 months, and those undergoing both types of SCT (auto-allo) 61 months; whereas for those who did not undergo SCT it was 28 months (p?=?0.845). The only statistically significant risk factor identified by multivariate analysis was hypercalcemia.

SUBMITTER: Nakaya A

PROVIDER: S-EPMC6043871 | biostudies-other | 2018

REPOSITORIES: biostudies-other

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Retrospective analysis of primary plasma cell leukemia in Kansai Myeloma Forum registry.

Nakaya Aya A Yagi Hideo H Kaneko Hitomi H Kosugi Satoru S Kida Toru T Adachi Yoko Y Shibayama Hirohiko H Kohara Takae T Kamitsuji Yuri Y Fuchida Shin-Ichi SI Uoshima Nobuhiko N Kawata Eri E Uchiyama Hitoji H Shimura Yuji Y Takahashi Takayuki T Urase Fumiaki F Ohta Kensuke K Hamada Tsuneyoshi T Miyamoto Kazue K Kobayashi Masayuki M Shindo Maki M Tanaka Hirokazu H Shimazaki Chihiro C Hino Masayuki M Kuroda Junya J Kanakura Yuzuru Y Takaoari-Kondo Akifumi A Nomura Shosaku S Matsumura Itaru I

Leukemia research reports 20180704

We retrospectively analyzed twenty-six patients with primary plasma cell leukemia (pPCL) registered from May 2005 until April 2015 by the Kansai Myeloma Forum. Twenty patients received novel agents (bortezomib or lenalidomide), and their median survival of was 34 months. The median survival of patients who underwent autologous stem cell transplantation (SCT) was 40 months, those undergoing allogeneic SCT 55 months, and those undergoing both types of SCT (auto-allo) 61 months; whereas for those w ...[more]

PMID: 30013912

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Project description:PurposePrimary plasma cell leukemia (pPCL) is an aggressive subtype of multiple myeloma, which is distinguished from newly diagnosed multiple myeloma (NDMM) on the basis of the presence of ≥ 20% circulating tumor cells (CTCs). A molecular marker for pPCL is currently lacking, which could help identify NDMM patients with high-risk PCL-like disease, despite not having been recognized as such clinically.MethodsA transcriptomic classifier for PCL-like disease was bioinformatically constructed and validated by leveraging information on baseline CTC levels, tumor burden, and tumor transcriptomics from 154 patients with NDMM included in the Cassiopeia or HO143 trials and 29 patients with pPCL from the EMN12/HO129 trial. Its prognostic value was assessed in an independent cohort of 2,139 patients with NDMM from the HOVON-65/GMMG-HD4, HOVON-87/NMSG-18, EMN02/HO95, MRC-IX, Total Therapy 2, Total Therapy 3, and MMRF CoMMpass studies.ResultsHigh CTC levels were associated with the expression of 1,700 genes, independent of tumor burden (false discovery rate < 0.05). Of these, 54 genes were selected by leave-one-out cross-validation to construct a transcriptomic classifier representing PCL-like disease. This not only demonstrated a sensitivity of 93% to identify pPCL in the validation cohort but also classified 10% of NDMM tumors as PCL-like. PCL-like MM transcriptionally and cytogenetically resembled pPCL, but presented with significantly lower CTC levels and tumor burden. Multivariate analyses in NDMM confirmed the significant prognostic value of PCL-like status in the context of Revised International Staging System stage, age, and treatment, regarding both progression-free (hazard ratio, 1.64; 95% CI, 1.30 to 2.07) and overall survival (hazard ratio, 1.89; 95% CI, 1.42 to 2.50).ConclusionpPCL was identified on the basis of a specific tumor transcriptome, which was also present in patients with high-risk NDMM, despite not being clinically leukemic. Incorporating PCL-like status into current risk models in NDMM may improve prognostic accuracy.

Dataset Information

Retrospective analysis of primary plasma cell leukemia in Kansai Myeloma Forum registry.

Publications

Retrospective analysis of primary plasma cell leukemia in Kansai Myeloma Forum registry.

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