Treatment of Multiple Myeloma Presenting as Primary Plasma Cell Leukaemia (PPCL)

Primary plasma cell leukaemia is a rare and particularly aggressive presentation of multiple myeloma in which large numbers of clonal plasma cells are detected directly in the peripheral blood — with no prior history of myeloma.

Clinical Scenario

This protocol applies to patients with primary plasma cell leukaemia (PPCL), defined by clonal plasma cells representing 20% or more of peripheral blood cells, or an absolute count exceeding 2 × 10⁹/l, arising in the absence of any previous multiple myeloma diagnosis. PPCL is operationally and clinically distinct from secondary PCL and carries a more aggressive disease course.

Treatment Approach

Management involves combination regimens that may include immunomodulatory agents and monoclonal antibodies, among other drug classes. Transplant eligibility is a key determinant of whether a continuous or time-limited treatment strategy is most appropriate. The specific regimen options, sequencing, and decision algorithm are available in the full structured protocol.

Instant Access to Structured Evidence-Based Regimens

References

Primary plasma cell leukaemia (PPCL) is a rare and aggressive variant of MM, operationally defined by the presence of 20% and/or an absolute number >2 × 10⁹/l of clonal plasma cells in the peripheral blood without a previous history of MM.
Patients not eligible for transplant procedures should preferably receive continuous treatment [III, C].
In relapsed/refractory PPCL, a switch to drugs not used at diagnosis should be considered, favouring combinations of lenalidomide or pomalidomide plus dexamethasone with K or mAbs (Dara or Elo) (expert consensus).

DOI: 10.1016/j.annonc.2020.11.014

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