多发性骨髓瘤向浆细胞白血病演变的知识现状

The Current State of Knowledge About Evolution of Multiple Myeloma to Plasma Cell Leukemia.

作者信息

Jurczyszyn Artur, Olszewska-Szopa Magdalena, Vesole David H

机构信息

Hematology Department, Jagiellonian University Medical College, Cracow, Poland.

Department of Hematology, Blood Neoplasms and Bone Marrow Transplantation, Wroclaw Medical University, Wroclaw, Poland.

出版信息

Clin Lymphoma Myeloma Leuk. 2023 Mar;23(3):188-193. doi: 10.1016/j.clml.2022.12.002. Epub 2022 Dec 6.

DOI:10.1016/j.clml.2022.12.002

PMID:36593169

Abstract

Plasma cell leukemia is a rare form of multiple myeloma (MM). In contrast to de novo primary plasma cell leukemia (pPCL), which is very uncommon presentation of MM, there is increasing frequency of transformation to secondary plasma cell leukemia (sPCL) with increasing survival of patients (MM). The molecular basis of sPCL remains poorly understood sPCL is particularly aggressive and is associated with an extremely poor prognosis, constituting a major unmet medical need. High-quality data in sPCL regarding presentation, treatment and outcomes is limited. Herein we review the current state of knowledge on sPCL diagnostics, molecular biology, clinical characteristics, prognosis and reported treatment outcomes and the emergence of the new therapeutic strategies.

摘要

浆细胞白血病是多发性骨髓瘤（MM）的一种罕见形式。与新发原发性浆细胞白血病（pPCL）不同，后者是MM非常罕见的表现形式，随着患者（MM）生存期的延长，转化为继发性浆细胞白血病（sPCL）的频率不断增加。sPCL的分子基础仍知之甚少。sPCL特别具有侵袭性，且预后极差，构成了一项重大的未满足医疗需求。关于sPCL的临床表现、治疗和结局的高质量数据有限。在此，我们综述了目前关于sPCL诊断、分子生物学、临床特征、预后、已报道的治疗结局以及新治疗策略出现的知识现状。