Plasma Cell Leukemia – Facts and Controversies: More Questions than Answers?

Authors:
  • ASAnna Suska
    Anna Suska
  • DVDavid H. Vesole
    David H. Vesole
  • JCJorge J. Castillo
    Jorge J. Castillo
  • SKShaji K. Kumar
    Shaji K. Kumar
  • HPHari Parameswaran
    Hari Parameswaran
  • MMMaria V. Mateos
    Maria V. Mateos
  • TFThierry Facon
    Thierry Facon
  • GMGabor Mikala
    Gabor Mikala
  • MSMarta Szostek
    Marta Szostek
  • JMJoseph Mikhael
    Joseph Mikhael
  • RHRoman Hajek
    Roman Hajek
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Abstract
Plasma cell leukemia (PCL) is an aggressive hematological malignancy characterized by an uncontrolled clonal proliferation of plasma cells (PCs) in the bone marrow and peripheral blood. PCL has been defined by an absolute number of circulating PCs exceeding 2.0 × 109/L and/or >20% PCs in the total leucocyte count. It is classified as primary PCL, which develops de novo, and secondary PCL, occurring at the late and advanced stages of multiple myeloma (MM). Primary and secondary PCL are clinically and biologically two distinct entities. After the diagnosis, treatment should be immediate and should include a proteasome inhibitor and immunomodulator-based combination regimens as induction, followed by stem cell transplantation (SCT) in transplant-eligible individuals who have cleared the peripheral blood of circulating PCs. Due to the rarity of the condition, there have been very few clinical trials. Furthermore, virtually all of the myeloma trials exclude patients with active PCL. The evaluation of response has been defined by the International Myeloma Working Group and consists of both acute leukemia and MM criteria. With conventional chemotherapy, the prognosis of primary PCL has been ominous, with reported overall survival (OS) ranging from 6.8 to 12.6 months. The use of novel agents and autologous SCT appears to be associated with deeper response and an improved survival, although it still remains low. The PCL prognostic index provides a simple score to risk-stratify PCL. The prognosis of secondary PCL is extremely poor, with OS of only 1 month.

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