Clincal Features And Prognosis Of Sixty Cases Of Waldenstr?m’s Macroglobulinemia

Background and ObjectivesWaldenstr?m macroglobulinemia is a type of lymphoproliferative disorder characterized by infiltration of small B lymphocytes,plasmacytoid lymphocytes and plasma cells in the bone marrow with increased monoclonal gammopath y of IgM in serum.The World Health Organization classifies Waldenstr?m mac-roglobulinemia as a lymphoplasmacytic lymphoma that secretes monoclonal Ig-M protein,which is a non-Hodgkin lymphoma.Most lymphoplasmacytic lymp-homas are Waldenstr?m macroglobulinemia,and less than 5%are composed o-f IgA type,IgG type,and non-secreted lymphoplasmacytic lymphoma.Although Waldenstr?m macroglobulinemia is still a clinically incurable disease,the rec-ent discovery of mutations in the MYD88 and CXCR4 genes further enhances-our understanding of its pathogenesis and brings new hope for treatment.As-ymptomatic Waldenstr?m macroglobulinemia patients do not need to treat.But people who have symptoms,such as hyperviscosity,neuropathy,condensed dise-ase,symptomatic organ enlargement,amyloidosis,cryoglobulinemia,disease re-lated cytopenia,lymph node enlargement,etc,should be treated.A number of clinical trials have been conducted in lymphoplasmacytic lymphoma/Waldenstr-?m macroglobulinemia abroad,but there are few clinical trials in China.This article retrospectively analyzed the data of 60 patients with symptomatic Wald-enstr?m macroglobulinemia diagnosed in our hospital,in order to investigate t-he clinical features,MYD88^L265P mutation,prognosis and treatment of symptomat-ic Waldenstr?m macroglobulinemia,which may provide a theoretical basis for the diagnosis and treatment of patients with symptomatic Waldenstr?m macrog-lobulinemia.MethodsSixty patients with symptomatic Waldenstr?m macroglobulinemia diagnosed of the First Affiliated Hospital of Zhengzhou University from February 1,2012 to July 1,2018 were choosed for retrospective analyzed,including clinical features,prognostic factors,ect.According to the mutation of MYD88 gene,it was divided into MYD88gene mutation group and wild group,and the clinical characteristics of the two groups were compared.Software SPSS21.0 was used for analyzing statistics.Differences between the two groups of continuous variables were analyzed by T test,and Fisher’s exact probability method was used to test whether there was statistical difference between the two groups.Kaplan-Meier was used for survival analysis,while the log-rank test was used for significant comparison.Cox regression test was used for multivariate survival analysis.Spearman rank correlation test was used for correlation analysis.P<0.05 was considered to be statistical significantly.ResultsAmong the 60 patients with WM,the male-female ratio was 2.53:1,and the med-ian age was 60.5（41-78）years.The anemia-related symptoms such as fatigue and di-zziness（33 cases,55%）were the main clinical manifestations.38（63.3%）had lymph-adenopathy and 52（87.0%）had IgMκtype.WPSS stratification:10 cases（16.7%）i-n the low-risk group,24 cases（40%）in the middle-risk group,and 26 cases（43.3%）i-n the high-risk group.Bone marrow flow cytology was performed in 55 patients,an-d 50（83.3%）were expressed as CD19+,CD20+,CD10-,CD5-,CD79+,FMC7+,CD23-.Of the 18 patients,14（77.8%）had MYD88L265P mutations,and the lower abs-olute value of lymphocytes（P<0.05）.Univariate analysis showed that albumin leve-l,lactate dehydrogenase level,IgA quantification,IgM quantification,and new drug c-hemotherapy were all associated with 5-year overall survival（OS）,and the differenc-e was statistically significant（P<0.05）.Cox multivariate analysis showed that albumi-n levels were independent risk factors for 5-year OS in WM patients,with HR（95%CI）of 8.632（1.912-38.983）.The median follow-up time for the entire group wa-s 13 months（2 months-92 months）,and the overall 5-year OS was 56.8%.ConclusionsThe main clinical features of patients with WM are anemia-related symptoms.l-ymphadenopathy is a common sign,and IgMκtype is the common type in W-M.The MYD88^L265P gene has a high incidence in WM and the group with MYD8-8^L265P had lower absolute value of lymphocytes than the wild group（P<0.05）.Albumi-n levels are independent risk factors that influence their prognosis.WM patients wh-o received a new drug regimen with rituximab,bortezomib,thalidomide,and lenalid-omide had longer 5-year OS than the conventional one.