Dubey Harshita, Goel Harsh, Verma Saransh, Gupta Swati, Tanwar Khushi, Rahul Ekta, Kapoor Gautam, Vasantharaman Jayashimman, Ranjan Amar, Tanwar Pranay, Chopra Anita
Laboratory Oncology Unit, Dr. B.R.A.-Institute Rotary Cancer Hospital, All India Institute of Medical Sciences New Delhi 110029, India.
Am J Blood Res. 2022 Dec 15;12(6):190-195. eCollection 2022.
Plasma cell leukemia (PCL) is a rare and aggressive plasma cell neoplasm distinguished by extensive clonal expansion of plasma cells in the bone marrow (BM) and peripheral blood (PB). PCL is divided into two subtypes: primary (pPCL) originates without preceding multiple myeloma, while secondary (sPCL) comprises a leukemic modification that occurs as a late manifestation from previous multiple myeloma (MM). pPCL and sPCL are clinically and biologically two different entities. The molecular mechanisms of the development of PCL, either primary or secondary, remain poorly understood. We aim to present 5 years of data on clinical profiles and treatment outcomes of pPCL and sPCL patients treated at our cancer hospital in India and to find a predictive parameter of the development of PCL in cases of MM.
In this study, we retrospectively reviewed and evaluated the clinicopathological features, laboratory parameters, immunophenotypic profile, and patient outcomes of 17 PCL cases diagnosed among 180 plasma cell dyscrasia patients during the study period to establish a correlation between pPCL & sPCL for diagnosis and management of PCL.
A total of 17 PCL patients were diagnosed among 180 plasma cell dyscrasia patients during the study period. Among PCL patients, 9 cases had pPCL (52.94% of all PCL patients), and 8 cases had sPCL (47.06% of all PCL patients). Peculiar differences were seen between the two PCL types. Both types of PCL had a younger age at the time of diagnosis, having elevated BM plasma cell infiltration percentage, frequent anemia, thrombocytopenia, elevated beta-2-microglobulin (B2M) levels, raised LDH levels, and positive M-protein in both serum and urine. In addition, SFLC assay and Immunofixation assay showed higher κ and lower λ in pPCL compared with sPCL (P<0.05). Higher Renal insufficiency was also observed in pPCL compared to sPCL (P=0.335). The survival and response to treatment of PCL patients remain considerably poor, sPCL exhibit shorter overall survival (OS) than pPCL with (median 1.75 months vs. 7 months respectively, ). Plasma cell leukemia (PCL) needs to be diagnosed early and requires prompt initiation of treatment before patients get complications.
Our study characterizes the clinical and laboratory features of pPCL and sPCL and may aid physicians in prognosticating the course of disease of their patients. However, future multicentre studies are the need of the hour to develop accurate diagnostic criteria and establish the efficacy of therapeutic regimens.
浆细胞白血病（PCL）是一种罕见且侵袭性的浆细胞肿瘤，其特征是骨髓（BM）和外周血（PB）中浆细胞广泛克隆性扩增。PCL分为两个亚型：原发性（pPCL）无前驱多发性骨髓瘤，而继发性（sPCL）是先前多发性骨髓瘤（MM）晚期出现的白血病性改变。pPCL和sPCL在临床和生物学上是两个不同的实体。原发性或继发性PCL发生发展的分子机制仍知之甚少。我们旨在呈现印度某癌症医院治疗的pPCL和sPCL患者5年的临床特征和治疗结果数据，并寻找MM病例中PCL发生发展的预测参数。
在本研究中，我们回顾性分析并评估了研究期间180例浆细胞异常增生患者中确诊的17例PCL患者的临床病理特征、实验室参数、免疫表型特征及患者预后，以建立pPCL和sPCL之间在PCL诊断和管理方面的相关性。
研究期间180例浆细胞异常增生患者中，共确诊17例PCL患者。在PCL患者中，9例为pPCL（占所有PCL患者的52.94%），8例为sPCL（占所有PCL患者的47.06%）。两种类型的PCL存在特殊差异。两种类型的PCL诊断时年龄均较轻，骨髓浆细胞浸润百分比升高，常伴有贫血、血小板减少、β2微球蛋白（B2M）水平升高、乳酸脱氢酶（LDH）水平升高，血清和尿液中M蛋白均呈阳性。此外，与sPCL相比，pPCL的血清游离轻链（SFLC）检测和免疫固定电泳显示κ更高而λ更低（P<0.05）。与sPCL相比，pPCL的肾功能不全发生率也更高（P=0.335）。PCL患者的生存及对治疗的反应仍然相当差，sPCL的总生存期（OS）比pPCL短（中位数分别为1.75个月和7个月）。浆细胞白血病（PCL）需要早期诊断，并在患者出现并发症之前迅速开始治疗。
我们的研究描述了pPCL和sPCL的临床及实验室特征，可能有助于医生预测患者的病程。然而，目前急需开展多中心研究以制定准确的诊断标准并确定治疗方案的疗效。
