Aug 2026· American journal of hematology/oncology· Vol 101, pp. 2853 - 2865· 0 citations· 111 references
Medicine
TL;DR
This review covers PCL biology, clinical features, prognosis, and treatment, emphasizing emerging therapies, especially BCMA‐directed CAR‐T, which show promise for deeper, more durable responses.
Abstract
Plasma cell leukemia (PCL) is a form of high‐risk multiple myeloma comprising 1%–2% of new myeloma diagnoses, and defined by ≥ 5% circulating plasma cells. Classified as primary, de novo or secondary, from relapsed/refractory myeloma, PCL carries worse outcomes than conventional myeloma despite advances. Genomically, PCL demonstrates high‐risk abnormalities like del(17p), 1q21 gain/amplification and overrepresentation of t(11;14), marking a distinct biological subgroup. Contemporary management involves quadruplet induction, early autologous stem cell transplantation in eligible patients, and multi‐agent consolidation/maintenance. Immune effector therapies, especially BCMA‐directed CAR‐T, show promise for deeper, more durable responses. This review covers PCL biology, clinical features, prognosis, and treatment, emphasizing emerging therapies.
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