Primary plasma cell leukemia 2.0: advances in biology and clinical management

ABSTRACT

Introduction: Primary plasma cell leukemia (PPCL) is a rare and aggressive variant of multiple myeloma. The introduction of novel agents and modern technologies has recently partially changed the clinical and biological scenario of this malignancy, allowing limited, but not negligible, progresses.

Areas covered: We will discuss: the complex landscape of genetic alterations in PPCL, derived from conventional and high-throughput technologies; the best available treatments for PPCL; the possible future therapeutic perspectives.

Expert commentary: PPCL requires an immediate and intensive multi-phase treatment with short therapy-free intervals, which should include novel agents and autologous stem cell transplantation in eligible patients. Allogeneic transplantation should be considered in selected cases. In older and/or frailer individuals, personalized approaches should be applied. Integrated treatments with next generation proteasome inhibitors/IMIDs and monoclonal antibodies are currently planned or under investigation. The identification of novel genomic biomarkers may be potentially helpful for risk stratification and future personalized therapies.

KEYWORDS:

• Plasma cell leukemia
• multiple myeloma
• molecular profiling
• risk stratification
• bortezomib
• lenalidomide
• stem cell transplantation
• proteasome inhibitors
• IMIDs
• monoclonal antibodies

Declaration of interest

P Musto has received honoraria and research funds from Janssen-Cilag and Celgene. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Additional information

Funding

This work was financially supported by the Associazione Italiana per la Ricerca sul Cancro to AN [IG 10136 and 16722] and the Italian Ministry of Health to KT [CUP Project E66110000230001] and to PM, VS, FN and GV [RRC-2015-2360451 and RRC-2016-2361129].