摘要
目的:探讨淋巴浆细胞淋巴瘤(LPL)患者的临床病理特征及生存情况。方法:回顾性分析2003年7月至2021年5月于大连医科大学附属第一医院住院的33例初治LPL患者资料,分析临床特点、骨髓细胞形态学、免疫分型、染色体核型、基因突变、治疗反应和预后,采用Kaplan-Meier法分析患者生存情况。结果:33例患者中位发病年龄66岁(55~84岁);男性26例(78.8%),女性7例(21.2%);常见的临床表现为贫血(31例,93.9%)、淋巴结肿大(16例,48.5%)和B症状(8例,24.2%)。所有患者均骨髓受累并伴M蛋白,其中23例(69.7%)IgM-κ型,8例(24.2%)IgM-λ型,1例(3.0%)IgG-κ型,1例(3.0%)IgA-κ型。骨髓涂片可见淋巴细胞、淋巴浆细胞或浆细胞增多;22例患者行骨髓流式细胞术免疫分型检查,患者均表达B细胞表面抗原(CD19和CD20),16例(72.7%)患者CD5、CD10阴性,13例(59.1%)CD138阳性或弱阳性,5例(22.7%)CD38阳性。23例行染色体检查的患者中7例(30.4%)染色体核型异常,16例行MYD88 L265P突变检查患者中14例(87.5%)突变阳性。21例可评估疗效患者中,18例(85.7%)治疗有反应,可达到部分缓解或疾病稳定,但完全缓解率低(14.3%,3/21)。中位随访34个月(2~102个月),1例失访;中位总生存时间未达到,3年和5年总生存率分别为79.2%和67.9%。结论:LPL是一类少见的好发于老年男性的惰性小B细胞淋巴瘤,病程较长,临床表现多样。血清学检查、骨髓细胞形态学及活组织检查、免疫分型和MYD88 L265P突变检测对于诊断及鉴别诊断具有重要意义。
Objective:To investigate the clinicopathological characteristics and survival of patients with lymphoplasmacytic lymphoma(LPL).Methods:The data of 33 newly diagnosed LPL patients in the First Affiliated Hospital of Dalian Medical University from July 2003 to May 2021 were retrospectively analyzed.The clinical characteristics,bone marrow cell morphology,immunophenotyping,chromosomal karyotype,gene mutation,treatment response and prognosis were analyzed,and Kaplan-Meier method was used to analyze the survival of patients.Results:The median age of onset of 33 patients was 66 years old(55-84 years old).There were 26 males(78.8%)and 7 females(21.2%).The common clinical manifestations were anemia(31 cases,93.9%),enlarged lymph nodes(16 cases,48.5%)and B symptoms(8 cases,24.2%).All patients had bone marrow involvement and M protein,23 of them(69.7%)were type IgM-κ,8 cases(24.2%)were type IgM-λ,1 case(3.0%)was type IgG-κ,and 1 case(3.0%)was type IgA-κ.Lymphocytes,lymphoplasmacytes or plasma cells was increased in bone marrow smear;22 patients underwent immunophenotyping of bone marrow by flow cytometry,and all patients expressed B cell surface antigens(CD19 and CD20),16 patients(72.7%)lost the expression of CD5 and CD10,13 patients(59.1%)expressed or weakly expressed CD138 and 5 patients(22.7%)expressed CD38.Seven out of 23 cases(30.4%)who received chromosome examination had abnormal chromosomal karyotype.Fourteen out of 16 cases(87.5%)who received MYD88 L265P mutation detection harbored the mutation.Among 21 patients with evaluable efficacy,18 patients(85.7%)responded to treatment,achieving partial remission or stable disease,but the rate of complete remission was low(14.3%,3/21).The median follow-up time was 34 months(2-102 months),1 case was lost.The median overall survival time was not reached,and the 3-year and 5-year overall survival rates were 79.2%and 67.9%,respectively.Conclusions:LPL is a rare indolent small B-cell lymphoma with a long course and a variety of manifestations,which is commonly seen in elderly
作者
申静枝
于家文
蔡薇
马亮亮
Shen Jingzhi;Yu Jiawen;Cai Wei;Ma Liangliang(Department of Hematology,the First Affiliated Hospital of Dalian Medical University,Dalian 11601l,China)
出处
《白血病．淋巴瘤》
CAS
2022年第10期593-598,共6页
Journal of Leukemia & Lymphoma
