肿瘤细胞治疗研究
英文原题：Primary Plasma Cell Leukemia.
Primary Plasma Cell Leukemia.
浆细胞白血病（PCL）是一种高危型多发性骨髓瘤，占新诊断骨髓瘤的1%-2%，其定义为循环浆细胞≥5%。
浆细胞白血病（PCL）是一种高危多发性骨髓瘤，占新诊断骨髓瘤的1%-2%，定义为循环浆细胞≥5%。根据来源可分为原发性、新发或继发性（由复发/难治性骨髓瘤转化而来），尽管治疗不断进步，PCL的预后仍差于传统骨髓瘤。在基因组学上，PCL表现出高危异常，如del(17p)、1q21获得/扩增以及t(11;14)的过度代表，标志着一个独特的生物学亚群。当代管理包括四联诱导治疗、适合患者的早期自体干细胞移植以及多药巩固/维持治疗。免疫效应疗法，尤其是靶向BCMA的CAR-T，显示出实现更深、更持久缓解的前景。本综述涵盖PCL的生物学、临床特征、预后和治疗，重点介绍新兴疗法。
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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