原发性浆细胞白血病33例诊疗分析

Diagnosis and treatment of thirty-three cases of plasma cell leukemia

目的分析原发性浆细胞白血病(pPCL)的临床特点、治疗和预后,以提高对该疾病的认识。方法回顾性分析2009年5月至2022年1月中国医科大学附属第一医院收治的33例pPCL患者的临床资料,分析其临床表现、实验室相关检查、治疗手段及预后。结果33例患者中,以乏力为最常见的首发症状,分型以IgGλ型最多见。同时表达CD38和CD138的患者占90.9%(20/22),复杂核型占70.6%(12/17)。19例患者接受化疗,1例行自体造血干细胞移植,总体缓解比例为9/19(47.4%)。结论恶性浆细胞疾病pPCL对常规传统化疗不敏感。蛋白酶体抑制剂和免疫调节剂组成的联合方案是目前较成熟的化疗方法,巩固治疗推荐造血干细胞移植。CAR-T、CD38单抗、Bcl-2抑制剂等新的治疗方式为患者带来新的生机。

Objective To analyze the clinical characteristics,treatment,prognosis and other aspects of primary plasma cell leukemia(pPCL),and improve the understanding of the disease.Methods A total of 33 patients with pPCL admitted to The First Hospital of China Medical University between May 2009 and January 2022 were retrospectively analyzed based on clinical manifestations,laboratory tests,treatment and prognosis.Results The most common initial symptom in all 33 patients was fatigue.IgGλwas the most common immune type.Most patients,20/22(90.9%)expressed both CD38 and CD138,and 12/17(70.6%)patients expressed complex karyotypes.Nineteen patients received chemotherapy,and one had an autologous stem cell transplantation.The overall remission rate was 9/19(47.4%).Conclusion As one of the malignant plasma cell diseases,pPCL is not sensitive to conventional chemotherapy.The combination of proteasome inhibitors and immunomodulatory drugs is the current,relatively advanced chemotherapy regimen,and hematopoietic stem cell transplantation is recommended for consolidation therapy.New therapeutics,such as CAR-T,CD38 monoclonal antibody and Bcl-2 inhibitors have brought new hope to patients.