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

Antonino Neri, Katia Todoerti, Marta Lionetti, Vittorio Simeon, Marzia Barbieri, Filomena Nozza, Gabriella Vona, Alessandra Pompa, Luca Baldini, Pellegrino Musto

Research output: Contribution to journalReview articlepeer-review


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.

Original languageEnglish
Pages (from-to)1063-1073
Number of pages11
JournalExpert Review of Hematology
Issue number11
Publication statusPublished - Nov 1 2016


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

ASJC Scopus subject areas

  • Hematology


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