Žárska M, Vrábel D, Bezdekova R, Štork M, Jarošová M, Adam Z, Krejčí M, Pour L, Ševčíková S
Klin Onkol. 2019 Winter;32(1):40-46.
Plasma cell leukemia (PCL) is a rare dis-ease and possibly the most aggressive form of monoclonal gammopathy. It is classified into two forms - primary PCL that occurs without a previously identifiable multiple myeloma stage, and secondary PCL that develops from previously dia-gnosed multiple myeloma. These two forms have different cytogenetic and molecular profiles, but both forms have an aggressive clinical course. Combinations of different therapeutic approaches includ-ing autologous stem cell transplantation and currently proteasome inhibitors and immunomodulatory drugs are used to treat PCL. Current dia-gnostic criteria, developed in the 1970s, may underestimate PCL prevalence; thus, prospective re-evaluation is be-ing considered.
The aim of this study is to review all available information about PCL with an emphasis on dia-gnostics, treatment, and circulat-ing plasma cells features.
Although PCL is rare, it is quite a severe dis-ease. Current treatments us-ing the latest therapeutics have prolonged patient survival. However, due to the low incidence of PCL, information about the dis-ease is very limited and comes mostly from small retrospective studies. Further studies of PCL are needed, because new information could increase in patient survival and our understand-ing of its pathogenesis. Key words plasma cell leukemia - multiple myeloma - plasma cells - cytogenetics - treatment This work was supported by grant NV18-03-00203. The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers. Submited: 2. 11. 2018 Accepted: 18. 11. 2018.
浆细胞白血病（PCL）是一种罕见疾病，可能是单克隆丙种球蛋白病最具侵袭性的形式。它分为两种类型——原发性PCL，即无前驱可识别的多发性骨髓瘤阶段而发生的PCL；以及继发性PCL，即由先前诊断的多发性骨髓瘤发展而来的PCL。这两种类型具有不同的细胞遗传学和分子特征，但都具有侵袭性的临床病程。包括自体干细胞移植以及目前的蛋白酶体抑制剂和免疫调节药物在内的不同治疗方法联合使用来治疗PCL。20世纪70年代制定的现行诊断标准可能低估了PCL的患病率；因此，正在考虑进行前瞻性重新评估。
本研究的目的是回顾关于PCL的所有现有信息，重点是诊断、治疗和循环浆细胞特征。
尽管PCL罕见，但却是一种相当严重的疾病。使用最新疗法的当前治疗方法延长了患者生存期。然而，由于PCL发病率低，关于该疾病的信息非常有限，且大多来自小型回顾性研究。需要对PCL进行进一步研究，因为新信息可能提高患者生存率并增进我们对其发病机制的理解。关键词：浆细胞白血病——多发性骨髓瘤——浆细胞——细胞遗传学——治疗 本研究得到了NV18 - 03 - 00203号资助。作者声明他们在研究中使用的药物、产品或服务方面不存在潜在利益冲突。编辑委员会声明该手稿符合ICMJE关于生物医学论文的推荐标准。提交日期：2018年11月2日 接受日期：2018年11月18日。
