Objective: To investigate the clinical characteristics, therapeutic response and prognosis of patients with plasma cell leukemia (PCL) and improve the understanding of this disease.
Methods: The clinical manifestations, laboratory tests and treatment response of 27 patients with plasma cell leukemia treated in The Second Hospital of Shanxi Medical University from December 2010 to August 2019 were analyzed retrospectively, and their clinical characteristics were summarized. Kaplan-Meier method was used for survival analysis.
Results: There were 18 cases of primary plasma cell leukemia (pPCL) and 9 cases of secondary plasma cell leukemia (sPCL). The male to female ratio was 1.7∶1. The median age was 62 years old. The first manifestations were bone pain, fatigue, fever, splenomegaly and bleeding, and a large number of plasma cell infiltration was observed in the morphological examination of peripheral blood and bone marrow cells. 13 cases were detected by immunotyping and all of them expressed CD38/CD138. 8 cases underwent karyotype analysis, and 3 cases were normal, clonal abnormalities occurred in 5 cases. FISH detection was performed in 12 cases, of which 8 cases were abnormal. In 17 cases of bortezomib based chemotherapy, the ovevall response rate was 52.9%, which was higher than that in the non-bortezomib group, but there was no significant difference between the two groups (P =0.242). The overall median survival time of 27 patients was 6.4 months, the median progression-free survival time was 3.5 months, and the median survival time of patients with pPCL and sPCL was 8.2 months and 2.4 months, respectively, the difference between the two groups was statistically significant (P =0.031).
Conclusion: PCL is highly invasive and has diverse clinical manifestations, and is not sensitive to traditional chemotherapy. The median survival time of patients with pPCL is relatively longer than that of patients with sPCL. The chemotherapy regimen based on bortezomib improves the treatment effectiveness and prolongs the survival time of PCL patients.
题目: 浆细胞白血病患者的临床特征及生存分析.
目的: 探讨浆细胞白血病（PCL）患者的临床特征、疗效反应及预后，提高对该病的认识。.
方法: 回顾性分析2010年12月至2019年8月山西医科大学第二医院收治的27例浆细胞白血病患者的临床表现、实验室检查及治疗反应，总结其临床特征，采用Kaplan-Meier法进行生存分析。.
结果: 原发性浆细胞白血病18例，继发性浆细胞白血病9例，男女比例为1.7∶1，中位年龄62岁，首发表现为骨痛、乏力、发热、脾大和出血，外周血及骨髓细胞形态学检查可见大量浆细胞浸润。13例行免疫分型检测，均表达CD38、CD138；8例行染色体核型检测，3例正常，5例出现克隆性异常改变；12例行FISH检测，其中8例异常。17例患者应用以硼替佐米为基础的化疗方案，总有效率为52.9%，高于非硼替佐米组，但两组比较差异无统计学意义（P =0.242）。27例患者总体中位生存期为6.4个月，中位无进展生存期为3.5个月；原发 性和继发性浆细胞白血病患者的中位生存期分别为8.2个月和2.4个月，差异具有统计学意义（P =0.031）。.
结论: 浆细胞白血病侵袭性强，临床表现多样，对传统化疗不敏感，原发性浆细胞白血病患者中位生存时间较继发性浆细胞白血病患者长，以硼替佐米为基础的化疗方案提高了治疗有效率，可从一定程度上延长浆细胞白血病患者的生存期。.
Keywords: bortezomib; clinical characteristics; plasma cell leukemia; prognosis; treatment response.