Objective:To explore the therapeutic efficacy and prognostic factors of combined rituximab and intensive chemotherapy for sporadic adult Burkitt lymphoma (BL) .Methods:This retrospective study examined the clinical and survival data of 30 patients newly diagnosed with BL between July 2011 and February 2023 at the Blood Diseases Hospital. Kaplan-Meier method was used for survival analysis, and the log-rank test was used for univariate analysis of prognostic factors.Results:The median age of the 30 patients was 43 years (24 - 66 years), and the male to female ratio was 3: 2. Extranodal invasion was present in 80% of the patients, with involvement of the bone marrow in 53.3% and central nervous system in 10.0%. The Ann Arbor stage was Ⅲ and Ⅳ in 86.7%. According to the number of Burkitt Lymphoma International Prognostic Index (BL-IPI) risk factors, patients were classified as low risk (0) in 20.0%, intermediate risk (1) in 43.3%, and high risk (≥2) in 36.7%. All patients were treated with an induction regimen of rituximab combined with intensive chemotherapy, with objective and complete response rates of 80.0% and 76.7%, respectively. The median follow-up was 49 months (6-153 months), and the 5-year progression-free survival (PFS) and overall survival (OS) rates were both (76.7±7.7) %. All patients with limited stage ( n=4) achieved continuous complete remission (CCR). Patients who had high risk, advanced stage sensitive to induction therapy ( n=10) sequentially received first-line autologous hematopoietic stem cell transplantation (auto-HSCT) as consolidation therapy; 9 patients achieved CCR, whereas 1 patient with central nervous system invasion developed early disease progression and died. The BL-IPI low, intermediate, and high risk groups had respective 5-year PFS rates of (83.3±15.2) %, 100.0%, and (45.5±15.0) % ( P=0.0069) and OS rates of (83.3±15.2) %, 100.0%, and (45.5±15.0) % ( P=0.0075). The main adverse effects of induction therapy were myelosuppression and secondary infections, which were effectively managed by appropriate symptomatic treatment. Univariate analysis demonstrated that worse PFS was associated with BL-IPI score ≥2 ( HR=4.90, 95% CI 1.02-23.45, P=0.0329) ; extranodal invasion at ≥2 sites ( HR=12.62, 95% CI 2.59-61.62, P=0.0021) ; and failure to achieve first complete response (CR1) after induction therapy ( HR=31.86, 95% CI 4.19-242.20, P<0.0001) . Conclusions:Intensive immunochemotherapy regimens were effective and well-tolerated by adult patients with highly aggressive BL. Treatment efficacy was ideal in patients with limited-stage disease, whereas prognosis was unsatisfactory in patients with high-risk BL-IPI. Sequential first-line auto-HSCT consolidation therapy may further improve outcomes in patients with high-risk advanced-stage disease who are sensitive to induction therapy. BL-IPI score ≥2, extranodal invasion at ≥2 sites, and failure to achieve CR1 after induction therapy were adverse prognostic factors in adult patients with BL.

Objective:To explore the therapeutic efficacy and prognostic factors of combined rituximab and intensive chemotherapy for sporadic adult Burkitt lymphoma (BL) .Methods:This retrospective study examined the clinical and survival data of 30 patients newly diagnosed with BL between July 2011 and February 2023 at the Blood Diseases Hospital. Kaplan-Meier method was used for survival analysis, and the log-rank test was used for univariate analysis of prognostic factors.Results:The median age of the 30 patients was 43 years (24 - 66 years), and the male to female ratio was 3: 2. Extranodal invasion was present in 80% of the patients, with involvement of the bone marrow in 53.3% and central nervous system in 10.0%. The Ann Arbor stage was Ⅲ and Ⅳ in 86.7%. According to the number of Burkitt Lymphoma International Prognostic Index (BL-IPI) risk factors, patients were classified as low risk (0) in 20.0%, intermediate risk (1) in 43.3%, and high risk (≥2) in 36.7%. All patients were treated with an induction regimen of rituximab combined with intensive chemotherapy, with objective and complete response rates of 80.0% and 76.7%, respectively. The median follow-up was 49 months (6-153 months), and the 5-year progression-free survival (PFS) and overall survival (OS) rates were both (76.7±7.7) %. All patients with limited stage ( n=4) achieved continuous complete remission (CCR). Patients who had high risk, advanced stage sensitive to induction therapy ( n=10) sequentially received first-line autologous hematopoietic stem cell transplantation (auto-HSCT) as consolidation therapy; 9 patients achieved CCR, whereas 1 patient with central nervous system invasion developed early disease progression and died. The BL-IPI low, intermediate, and high risk groups had respective 5-year PFS rates of (83.3±15.2) %, 100.0%, and (45.5±15.0) % ( P=0.0069) and OS rates of (83.3±15.2) %, 100.0%, and (45.5±15.0) % ( P=0.0075). The main adverse effects of induction therapy were myelosuppression and secondary infections, which were effectively managed by appropriate symptomatic treatment. Univariate analysis demonstrated that worse PFS was associated with BL-IPI score ≥2 ( HR=4.90, 95% CI 1.02-23.45, P=0.0329) ; extranodal invasion at ≥2 sites ( HR=12.62, 95% CI 2.59-61.62, P=0.0021) ; and failure to achieve first complete response (CR1) after induction therapy ( HR=31.86, 95% CI 4.19-242.20, P<0.0001) . Conclusions:Intensive immunochemotherapy regimens were effective and well-tolerated by adult patients with highly aggressive BL. Treatment efficacy was ideal in patients with limited-stage disease, whereas prognosis was unsatisfactory in patients with high-risk BL-IPI. Sequential first-line auto-HSCT consolidation therapy may further improve outcomes in patients with high-risk advanced-stage disease who are sensitive to induction therapy. BL-IPI score ≥2, extranodal invasion at ≥2 sites, and failure to achieve CR1 after induction therapy were adverse prognostic factors in adult patients with BL.

Objective:To explore the efficacy and safety of ibrutinib for the treatment of newly treated and relapsed refractory (R/R) lymphoplasmacytic lymphoma (LPL) /Waldenstr?m macroglobulinemia (WM) .Methods:Retrospectively collected clinical data of 98 cases of newly treated and R/R LPL/WM patients who received ibrutinib treatment at the Hematology & Blood Diseases Hospital of the Chinese Academy of Medical Sciences from March 2016 to June 2023, and analyzed their efficacy and safety.Results:A total of 98 LPL/WM patients were included, which consisted of 45 newly treated patients and 53 R/R patients. Of these, 74 were males (75.5%) and the cohort had a median age of 64 (42-87) years. Eighty-eight patients were eligible for efficacy evaluation with a median treatment time of 20.8 (2.1-55.0) months, a major remission rate (MRR) of 78.4%, and an overall response rate (ORR) of 85.2%. The MRR and ORR of the newly treated patients were 78.4% and 86.5%, respectively, whereas the MRR and ORR of the R/R patients were 78.4% and 84.3%, respectively. There were no statistically significant differences in MRR and ORR between the initial treatment and R/R patients (all P values >0.05) . The median follow-up period was 29.1 (2.9-50.3) months and the median overall survival time for newly treated and R/R patients was not reached. The median progression-free survival time was 23.5 (95% CI 10.5-36.5) months and 45.0 (95% CI 34.0-56.0) months, respectively, with no statistically significant differences (all P values >0.05) . There were 25 deceased patients and no deaths were related to ibrutinib treatment. The main adverse reactions of ibrutinib were thrombocytopenia (5.1%) , pneumonia (8.1%) , and hyperuricemia (21.4%) . The incidence of atrial fibrillation was 2.0%. Conclusion:Ibrutinib exhibits good efficacy and safety for newly treated and R/R LPL/WM patients.

Objective:This study aimed to summarize the clinical characteristics and prognosis of patients with bone marrow invasive follicular lymphoma (FL) and discuss the treatment modalities.Methods:This study included 183 consecutive patients with FL accompanied by bone marrow invasion and receiving regular treatment at the Hospital of Hematology, Chinese Academy of Medical Sciences, from January 2013 to December 2022. Clinical data were retrospectively collected and analyzed, and single and multifactorial analyses of survival prognosis were conducted with the Kaplan-Meier method and Cox regression model.Results:The median age was 48 (range: 19 - 78) years, and the male-to-female ratio was 0.9∶1. All of the patients had bone marrow invasion, 27.8% had increased lactate dehydrogenase levels, 42.1% had lymphocyte counts of >5×10 9/L, 18.4% had abnormal chromosomal karyotypes, and 48.6% had Ki-67 index of ≥30% in lymphoid tissue. Comparison of different subgroups: lymphocyte counts of >5×10 9/L, number of lymph nodes of ≥5 involved, and proportion of bone marrow chromosomal abnormalities occurring were higher in the anthracycline-intensive treatment group than in the rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) protocol and the nucleoside analog (including CD20 monoclonal antibody in combination with fludarabine and bendamustine) groups (all P<0.05). The complete remission rate was 39.1% in the conventional R-CHOP group, which was lower and statistically significant than that in the intensive treatment group (55.1%) and the nucleoside analog group (62.5%) ( P=0.042). The multivariate analysis for survival analysis revealed high risk of FLIPI ( HR= 1.910, 95% CI 1.036 - 3.522, P=0.036), chromosomal abnormalities karyotype ( HR=2.666, 95% CI 1.333-5.331, P=0.006), and conventional R-CHOP treatment ( HR=2.287, 95% CI 1.140-4.591, P=0.020) were the independent adverse prognostic factors affecting progression-free survival (PFS), whereas POD24 was the only independent adverse prognostic factor affecting overall survival (OS) adverse prognostic factor ( HR=9.581, 95% CI 3.000 - 30.593, P<0.001) . Conclusions:The clinical presentations of patients with bone marrow invasive FL were easy to combine the clinical features, including increased lymphocyte count, chromosomal abnormalities, and Ki-67 index in lymphoid tissues. The FLIPI score, chromosomal abnormal karyotype, and high-lymphoid-tissue Ki-67 index were the poor prognostic factors influencing PFS. R-CHOP therapy demonstrated a poor prognosis in this group of patients.

Objective:This study aimed to summarize the clinical characteristics and prognosis of patients with bone marrow invasive follicular lymphoma (FL) and discuss the treatment modalities.Methods:This study included 183 consecutive patients with FL accompanied by bone marrow invasion and receiving regular treatment at the Hospital of Hematology, Chinese Academy of Medical Sciences, from January 2013 to December 2022. Clinical data were retrospectively collected and analyzed, and single and multifactorial analyses of survival prognosis were conducted with the Kaplan-Meier method and Cox regression model.Results:The median age was 48 (range: 19 - 78) years, and the male-to-female ratio was 0.9∶1. All of the patients had bone marrow invasion, 27.8% had increased lactate dehydrogenase levels, 42.1% had lymphocyte counts of >5×10 9/L, 18.4% had abnormal chromosomal karyotypes, and 48.6% had Ki-67 index of ≥30% in lymphoid tissue. Comparison of different subgroups: lymphocyte counts of >5×10 9/L, number of lymph nodes of ≥5 involved, and proportion of bone marrow chromosomal abnormalities occurring were higher in the anthracycline-intensive treatment group than in the rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) protocol and the nucleoside analog (including CD20 monoclonal antibody in combination with fludarabine and bendamustine) groups (all P<0.05). The complete remission rate was 39.1% in the conventional R-CHOP group, which was lower and statistically significant than that in the intensive treatment group (55.1%) and the nucleoside analog group (62.5%) ( P=0.042). The multivariate analysis for survival analysis revealed high risk of FLIPI ( HR= 1.910, 95% CI 1.036 - 3.522, P=0.036), chromosomal abnormalities karyotype ( HR=2.666, 95% CI 1.333-5.331, P=0.006), and conventional R-CHOP treatment ( HR=2.287, 95% CI 1.140-4.591, P=0.020) were the independent adverse prognostic factors affecting progression-free survival (PFS), whereas POD24 was the only independent adverse prognostic factor affecting overall survival (OS) adverse prognostic factor ( HR=9.581, 95% CI 3.000 - 30.593, P<0.001) . Conclusions:The clinical presentations of patients with bone marrow invasive FL were easy to combine the clinical features, including increased lymphocyte count, chromosomal abnormalities, and Ki-67 index in lymphoid tissues. The FLIPI score, chromosomal abnormal karyotype, and high-lymphoid-tissue Ki-67 index were the poor prognostic factors influencing PFS. R-CHOP therapy demonstrated a poor prognosis in this group of patients.

Humans ; Waldenstrom Macroglobulinemia/genetics* ; East Asian People ; Chromosome Aberrations ; Lymphoma

Humans ; Prognosis ; Multiple Myeloma/diagnosis* ; Neoplasm Staging ; Hematopoietic Stem Cell Transplantation ; Patients ; Retrospective Studies

Objective:To investigate the clinical and pathological features, treatment, and prognosis of gray zone lymphoma (GZL) .Methods:From July 2, 2013, to February 10, 2021, the clinical and pathological features, treatment, and outcomes of five patients with GZL at the Blood Diseases Hospital, Chinese Academy of Medical Sciences were studied retrospectively.Results:There were one male and 4 females, with a median age of 28 (16-51) years at diagnosis. Four patients had mediastinal (thymic) involvement, two of which had superior vena cava obstruction syndrome, and 3 patients had extra-nodal involvement. There was one case with a limited Ann Arbor stage and 4 cases with a progressive stage. Three patients had cHL-like pathomorphology with scattered Hodgkin-like cells, strongly positive for CD20, positive for CD30, and CD15 was negative; the other two patients had both cHL and DLBCL morphology, with some areas resembling Hodgkin cells and some areas resembling immunoblasts, strongly positive for CD30, and CD15 but negative CD20. Two patients were treated with cHL-like regimens for induction and achieved only partial remission; after salvage therapy with enhanced DLBCL-like regimens, all achieved complete remission (CR) . Three patients were treated with enhanced DLBCL-like immunochemotherapy regimens for induction, and two patients were effective, one of whom achieved CR. Four patients who did not achieve CR were given second or third-line salvage therapy, and all of them recovered. One patient lost parity, one died of disease progression at 35.9 months after diagnosis, and the remaining three maintained sustained remission.Conclusions:GZL is uncommon, usually affects younger patients, is mediastinal and is diagnosed using path morphology and immunophenotype. Patients with newly diagnosed GZL appear to be more sensitive to DLBCL-like immunochemotherapy regimens; relapsed or refractory patients were tended with non-cross-resistant combination chemotherapy or with new drugs.