Babu K Govind, Jacob Linu Abraham, Agarwal Ankit, Lakshmaiah K C, Lokanatha D, Suresh Babu M C, Rajeev L K, Lokesh K N, Rudresha A H
Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India.
Indian J Cancer. 2016 Oct-Dec;53(4):619-620. doi: 10.4103/0019-509X.204775.
The first case of plasma cell leukemia (PCL) was recognized by Gluzinski and Reichentein. It is the most aggressive among the monoclonal gammopathies. It is diagnosed by the presence of more than 20% plasma cells in the peripheral blood or an absolute plasma cell count of> 2000/mm3. Because of the relatively low incidence, most data come from case reports and retrospective studies. No prospective series have been published, and only seven reports including more than twenty patients have been identified. We report a retrospective series of 18 patients identified as PCL.
To study the clinical features and outcome of patients with PCL.
A retrospective study was conducted from the year 2006 to 2015 wherein all the patients diagnosed with PCL were identified. Complete clinical and treatment details and outcome were obtained from the records.
There were total 18 cases of PCL (3.7% of cases with multiple myeloma) diagnosed between the year 2006 and 2015. 16 cases (84%) were primary PCL, and two cases were secondary PCL. Twelve patients were males and six were females. The median age was 56.5 years. All patients had aggressive clinical course and median overall survival even with immunomodulatory agents was only 3 months.
PCL is a very aggressive disease, and no prospective trials have been conducted. Patients with PCL require induction with immunomodulators, proteasome inhibitors, and further trials are needed to evaluate the role of autologous stem cell transplant in this disease.
浆细胞白血病(PCL)的首例病例由格鲁津斯基和赖兴泰因确认。它是单克隆丙种球蛋白病中最具侵袭性的。外周血中浆细胞超过20%或绝对浆细胞计数>2000/mm³可诊断该病。由于发病率相对较低,大多数数据来自病例报告和回顾性研究。尚无前瞻性系列研究发表,仅发现七篇包含二十多名患者的报告。我们报告了一组18例确诊为PCL的回顾性病例。
研究PCL患者的临床特征和预后。
对2006年至2015年期间确诊为PCL的所有患者进行回顾性研究。从记录中获取完整的临床、治疗细节及预后情况。
2006年至2015年期间共确诊18例PCL(占多发性骨髓瘤病例的3.7%)。16例(84%)为原发性PCL,2例为继发性PCL。12例为男性,6例为女性。中位年龄为56.5岁。所有患者临床病程均具侵袭性,即使使用免疫调节剂,中位总生存期也仅3个月。
PCL是一种极具侵袭性的疾病,尚无前瞻性试验。PCL患者需要用免疫调节剂、蛋白酶体抑制剂进行诱导治疗,还需要进一步试验来评估自体干细胞移植在该病中的作用。