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Patterns of Progression and Clinical Outcomes After First Progression in Patients With Primary Plasma Cell Leukemia
Daniel G Packard1, Shaji K Kumar1, Angela Dispenzieri1
1Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
American Journal of Hematology
|June 4, 2026
Summary
Primary plasma cell leukemia (pPCL) is aggressive, with outcomes worsening after initial treatment. Novel therapies like anti-BCL-2 and CAR T-cell show promise for managing disease progression in pPCL patients.
Area of Science:
- Hematology
- Oncology
- Clinical Research
Background:
- Primary plasma cell leukemia (pPCL) is a rare, aggressive hematologic malignancy.
- It presents with inferior outcomes compared to multiple myeloma (MM).
- Limited data exists on outcomes following first progression and subsequent therapies in pPCL.
Purpose of the Study:
- To retrospectively analyze treatment outcomes in patients with primary plasma cell leukemia (pPCL) after first-line therapy progression.
- To assess the impact of disease progression type (symptomatic vs. biochemical) on overall survival (OS).
- To evaluate the efficacy of novel therapeutic approaches in advanced pPCL.
Main Methods:
- Retrospective analysis of 104 pPCL patients treated between 2006-2024.
- Kaplan-Meier methods used to assess time to next treatment (TTNT) and overall survival (OS).
- Evaluation of clinical characteristics, treatments, and cytogenetic risk factors.
Main Results:
- Median OS was 39 months; 71% had high-risk cytogenetics.
- Median TTNT after first-line therapy was 10 months, shortening with subsequent lines.
- Symptomatic progression was linked to significantly inferior OS (13 vs. 34 months).
- Anti-BCL-2 therapy in t(11;14) pPCL and anti-BCMA CAR T-cell therapy demonstrated improved TTNT (13 and 32 months, respectively).
Conclusions:
- Primary plasma cell leukemia (pPCL) is characterized by frequent progression and decreasing response durations.
- Symptomatic progression is a critical negative prognostic factor in pPCL.
- Targeted therapies including anti-BCL-2 agents and anti-BCMA CAR T-cells offer improved disease control in advanced pPCL.
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