非IgM型淋巴浆细胞淋巴瘤临床及生物学特征

Clinical and biological characteristics of non-IgM lymphoplasmacytic lymphoma

目的探索非IgM型淋巴浆细胞淋巴瘤(LPL)患者的临床及生物学特征。方法回顾性收集中国医学科学院血液病医院1993年7月至2020年8月收治的340例LPL患者的临床资料, 其中23例为非IgM型LPL组, 317例为华氏巨球蛋白血症(WM)组。比较两组患者的临床及生物学特征。结果 23例非IgM型LPL患者中, 2例分泌单克隆性IgA, 14例分泌单克隆性IgG, 7例不分泌单克隆性免疫球蛋白。非IgM型LPL和WM患者中位年龄均为62(35~81)岁。与WM组患者相比, 非IgM型LPL组患者女性(56.5%对27.3%, P=0.007)、脾大(60.1%对43.8%, P=0.100)、结外侵犯(21.7%对12.3%, P=0.672)比例更高。非IgM型LPL组18例患者进行了MYD88基因相关检测, 阳性率55.6%。非IgM型LPL组17例患者接受了治疗, 启动治疗的患者比例与WM组患者相当(94.4%对92.7%, P=0.488)。非IgM型LPL组16例患者进行了疗效评价, 一线治疗总体缓解率87.5%, 中位随访时间33.9(3.5~125.1)个月, 总体中位无进展生存(PFS)、总生存(OS)时间未达到, 3年PFS率和OS率分别为71.4%和68.9%。WM组中位PFS、OS时间分别为66.2个月和78.1个月。两组PFS、OS的差异均无统计学意义(P值分别为0.340、0.544)。结论非IgM型LPL与WM患者的临床及生物学特征相似, 但非IgM型LPL组女性、结外受累比例更高。非IgM型LPL患者的生存及预后与WM患者相似。

Objective The study aims to explore the clinical and biological characteristics of patients with non-IgM lymphoplasmacytic lymphoma(LPL).Methods The clinical data of 340 patients with LPL admitted to the Institute of Hematology&Blood Diseases Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College were collected retrospectively,including 23 cases of the non-IgM LPL and 317 cases of the Waldenström's macroglobulinemia(WM),from July 1993 to August 2020.The clinical and biological characteristics of the two groups were compared.Results Among 23 patients with the non-IgM type LPL,two patients secreted monoclonal IgA,14 patients secreted monoclonal IgG,and seven patients did not secrete monoclonal immunoglobulin.The median age of the non-IgM LPL and WM were both 62(35-81)years old.Compared with the WM group,the proportion of women(56.5%vs 27.3%,P=0.007),the proportion of splenomegaly(60.1%vs 43.8%,P=0.100),and the proportion of extranodal invasion(21.7%vs 12.3%,P=0.672)in non-IgM LPL group were higher.Eighteen patients were tested for MYD88 gene mutation,and the overall mutation rate of MYD88 was 55.6%.In the non-IgM LPL group,a total of 17 patients received treatment,which had a comparable proportion(94.4%vs 92.7%,P=0.488)to the WM group.Sixteen patients were evaluated for efficacy,and the overall remission rate of the first-line treatment was 87.5%.The median follow-up time was 33.9(3.5-125.1)months,and the median PFS and OS were both not reached.The 3-year PFS and OS rates were 71.4%and 68.9%,respectively.In the WM group,the median PFS was 66.2 months and the median OS was 78.1 months.Compared with the WM group,in the non-IgM group no significant differences in PFS(P=0.340)and OS(P=0.544)were seen.Conclusion The clinical and biological characteristics of the non-IgM LPL and WM patients were similar.However,the proportion of women and extranodal involvement were higher in the non-IgM LPL group.The survival and prognosis of the non-IgM LPL patients were similar to those of the WM patients.