Primary plasma cell leukaemia (PPCL) is a rare and aggressive presentation of multiple myeloma with a distinct treatment approach driven by the severity and urgency of the disease.
This protocol applies to patients with primary plasma cell leukaemia (PPCL) — defined by clonal plasma cells present in the peripheral blood at ≥20% or an absolute count of >2 × 109/l, with no prior history of multiple myeloma. PPCL is operationally distinct from secondary plasma cell leukaemia and requires immediate, intensive intervention.
Given the aggressive biology, treatment must begin promptly. The approach involves multiphase therapy incorporating proteasome inhibitor– and/or immunomodulatory agent–based combinations together with chemotherapy agents, typically spanning induction through to transplant-based consolidation and ongoing maintenance. Additional transplant strategies, including allogeneic stem cell transplantation in carefully selected patients, may factor into the overall plan.
The complete regimen selection, sequencing, eligibility criteria, and all dosing details are available in the full structured protocol.
DOI: 10.1016/j.annonc.2020.11.014
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.
Overall, treatment should be immediate and possibly oriented toward bortezomib and/or lenalidomide-based multiphase approaches in combination with chemotherapy agents, with short treatment-free intervals.
It should ideally include induction, double ASCT, consolidation and maintenance [II, B]. Allo-SCT should be considered in selected cases [III, C].
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