How I Treat Plasma Cell Leukemia.

Area of Science:

• Hematology
• Oncology
• Molecular Biology

Background:

• Plasma cell leukemia (PCL) is a rare and aggressive plasma cell malignancy.
• It is defined by ≥5% circulating plasma cells in peripheral blood and associated with high-risk features.

Purpose of the Study:

• To review current evidence and clinical approaches for PCL.
• To highlight knowledge gaps and the need for dedicated clinical trials.

Main Methods:

• Literature review of current evidence on PCL.
• Synthesis of data from multiple myeloma (MM) trials and novel therapeutic studies.
• Presentation of illustrative clinical cases.

Main Results:

• PCL exhibits distinct clinical and molecular features, including extramedullary involvement and high-risk cytogenetics.
• Novel therapies like BCMA-directed treatments, GPRC5D-targeted therapy, and BCL-2 inhibition show promise.
• Current treatment strategies are often extrapolated from MM, with PCL patients largely excluded from trials.

Conclusions:

• Despite advances, PCL remains inadequately studied.
• Dedicated prospective clinical trials are crucial to improve outcomes for PCL patients.
• Novel therapeutic strategies offer hope but require further investigation in dedicated PCL studies.