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Primary Plasma Cell Leukemia
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DOI：10.1002/ajh.70487.png)
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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.
Keyword:
circulating plasma cells
high-risk myeloma
multiple myeloma
plasma cell leukemia
期刊
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9.9
论文数:
8.6K
被引数:
1.7W
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