A Clinical Study On Patients With Waldenstr(?)m's Macroglobulinemia

ObjectiveTo investigate the clinical characteristics and prognostic factors of Waldenstr(?)m's macroglobulinemia(WM)patients from China.MethodsClinical data of 24 patients with WM in Zhengzhou University People's Hospital from January 2011 to January 2016 were analyzed retrospectively.ResultsAmong the 24 cases,the ratio of male to female was 2:1(16 males and 8 females).The median age was 62.5(42-79)years old.And the most common symptom was fatigue(20 cases,83.3%).The median hemoglobin level was 75(46-145)g/L.The median IgM level was 32.9(6.4-79.3)g/L.18 cases(75%)secreted kappa light chain and 6 cases(25%)secreted lambda light chain.8 patients underwent bone marrow biopsy,including 3 cases of reticular fiber staining for(++~+++).In 16 patients with available immunophenotypic data,13 cases(81.3%)expressed sIgM,CD19,CD20 and CD22,but missed the expression of CD5,CD10 and CD23.The median progression-free survival(PFS)were 7.5(1-51)months.Univariate analysis revealed that many factors influenced PFS of WM patients,including age,hemoglobin,platelet,IgM concentration,albumen,?2 microglobulin,C-reactive protein,lactate dehydrogenase,serum creatinine(SCr)and severe immune paralysis.Patients with the application of chemotherapy regimens containing rituximab or bortezomib had longer PFS than those who did not apply.Based on Cox multivariate analysis,the independent adverse prognostic factors included age(P=0.008),IgM concentration(P=0.028),and SCr(P=0.005).Using age,IgM concentration and SCr,three different prognostic groups could been distinguished,with median PFS of 26 months,6 months and 2 months(P<0.001).ConclusionsWM often occurs in elderly men and secretes kappa light chain.WM has diverse and non-specifice clinical manifestations.Chinese WM patients have distinct characteristics,such as younger age and more severe clinical manifestations.The clinical course of WM is indolent B-cell lymphoma.The complication of myelofibrosis in WM may occur.The prognostic system based on age,IgM concentration,and SCr may well predict the survival of WM patients from China.The application of rituximab or bortezomib is expected to improve curative effect and prognosis.