Objective: To analyze and compare therapy responses, outcomes, and incidence of severe hematologic adverse events of flumatinib and imatinib in patients newly diagnosed with chronic phase chronic myeloid leukemia (CML) . Methods: Data of patients with chronic phase CML diagnosed between January 2006 and November 2022 from 76 centers, aged ≥18 years, and received initial flumatinib or imatinib therapy within 6 months after diagnosis in China were retrospectively interrogated. Propensity score matching (PSM) analysis was performed to reduce the bias of the initial TKI selection, and the therapy responses and outcomes of patients receiving initial flumatinib or imatinib therapy were compared. Results: A total of 4 833 adult patients with CML receiving initial imatinib (n=4 380) or flumatinib (n=453) therapy were included in the study. In the imatinib cohort, the median follow-up time was 54 [interquartile range (IQR), 31-85] months, and the 7-year cumulative incidences of CCyR, MMR, MR(4), and MR(4.5) were 95.2%, 88.4%, 78.3%, and 63.0%, respectively. The 7-year FFS, PFS, and OS rates were 71.8%, 93.0%, and 96.9%, respectively. With the median follow-up of 18 (IQR, 13-25) months in the flumatinib cohort, the 2-year cumulative incidences of CCyR, MMR, MR(4), and MR(4.5) were 95.4%, 86.5%, 58.4%, and 46.6%, respectively. The 2-year FFS, PFS, and OS rates were 80.1%, 95.0%, and 99.5%, respectively. The PSM analysis indicated that patients receiving initial flumatinib therapy had significantly higher cumulative incidences of CCyR, MMR, MR(4), and MR(4.5) and higher probabilities of FFS than those receiving the initial imatinib therapy (all P<0.001), whereas the PFS (P=0.230) and OS (P=0.268) were comparable between the two cohorts. The incidence of severe hematologic adverse events (grade≥Ⅲ) was comparable in the two cohorts. Conclusion: Patients receiving initial flumatinib therapy had higher cumulative incidences of therapy responses and higher probability of FFS than those receiving initial imatinib therapy, whereas the incidence of severe hematologic adverse events was comparable between the two cohorts.
Adult ; Humans ; Adolescent ; Imatinib Mesylate/adverse effects* ; Incidence ; Antineoplastic Agents/adverse effects* ; Retrospective Studies ; Pyrimidines/adverse effects* ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive/drug therapy* ; Treatment Outcome ; Benzamides/adverse effects* ; Leukemia, Myeloid, Chronic-Phase/drug therapy* ; Aminopyridines/therapeutic use* ; Protein Kinase Inhibitors/therapeutic use*

OBJECTIVE: To explore the characteristics of ADC value changes in DWI of newly diagnosed symptomatic MM patients and its correlation with R-ISS stage. METHODS: The data of 148 newly diagnosed symptomatic MM patients treated by whole-body DWI scan at The First Affiliated Hospital of Soochow University from June 2016 to June 2019 were selected and retrospectively analyzed and 30 cases of age-matched healthy people were selected as controls. The differences of ADC values between the patients in normal control group, DWI^- group and DWI⁺ group were compared, and the relationship between ADC values and R-ISS stage in MM patients was compared. RESULTS: The plasma cell percentage of the patients in DWI⁺ group was higher than those in DWI^- group. ADC values of vertebra, sternum, rib, pectoral girdle, pelvic girdle of the patients in DWI⁺ group were significantly higher than those in DWI^- group and normal control group. The ADC values of each part of the patients in DWI^- group were higher than those in normal control group. ADC values of sternum, rib and pectoral girdle in the patients at R-ISS stage III were higher than those at R-ISS stage I and II, while, there was no statistical difference between R-ISS stage I and II groups. And there was no significant difference in ADC values of other bone parts such as vertebra and pelvic girdle in patients at R-ISS stage Ⅰ-Ⅲ. CONCLUSION DWI⁺ in MM patients is related to higher tumor invasion. The ADC values of the DWI⁺ group are higher than those of the DWI^- group; the bone ADC values of the DWI^- patients are still higher than the normal ones. And there is a certain relationship between ADC value and R-ISS stage.
Bone Diseases ; Diffusion Magnetic Resonance Imaging ; Humans ; Multiple Myeloma/diagnostic imaging* ; Retrospective Studies ; Whole Body Imaging

OBJECTIVE: To observe the detection of fusion gene in children with acute lymphoblastic leukemia (ALL) of different immunophenotypes, and analyze the relationship between fusion gene and prognosis. METHODS: The clinical data of 86 children with ALL treated in the hospital from May 2015 to May 2020 were retrospectively analyzed, the immunophenotypes and the prognosis of children were recorded, the detection of fusion gene in ALL children with different immunophenotypes was compared, the relationship between detection of fusion gene and prognosis was analyzed. RESULTS: The results of bone marrow immunophenotype showed that there were 13 cases of T cell type and 73 cases of B cell type in 86 children with ALL. The detection rate of fusion gene SIL-TAL1 in ALL children with T cell type was significantly higher than that in ALL children with B cell type (P<0.05). The detection rates of fusion genes BCR-ABL1, E2A-PBX1 and TEL-AML1 in ALL children with B cell type were higher than those in ALL children with T cell type, but the differences were not statistically significant (P>0.05). Followed up for 8-12 months, recurrence was taken as the end point, the average follow-up time was (10.14±1.75) months, in 86 children with ALL 15 cases recurred (17.44%). The recurrence curve drawn by Kaplan-Meier method showed that the median recurrence time of 15 children with recurrent ALL was 9 months. The proportions of positive minimal residual disease and extramedullary infiltration in the poor prognosis group were higher than those in the good prognosis group, and the differences were statistically significant (P<0.05). The detection rates of fusion genes BCR-ABL and SIL-TAL1 in the poor prognosis group were higher than those in the good prognosis group, and the differences were statistically significant (all P<0.05). Logistic regression analysis showed that positive minimal residual lesions, extramedullary infiltration, and detection of fusion genes BCR-ABL and SIL-TAL1 were risk factors for poor prognosis in children with ALL (OR>1, P<0.05). The ROC curve analysis showed that the area under the curve (AUC) of combined detection of fusion gene BCR-ABL and SIL-TAL1 for predicting the poor prognosis of ALL children was >0.707, which had a certain predictive value. CONCLUSION There are differences in fusion genes among ALL children with different immunophenotypes, minimal residual disease, extramedullary infiltration, and fusion gene are associated with prognosis of ALL children. Fusion gene detection can be used as new method to predict the prognosis of children with ALL.
Child ; Humans ; Neoplasm, Residual ; Retrospective Studies ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics* ; Oncogene Proteins, Fusion/genetics*

OBJECTIVE: To investigate the clinical effect and safety of Chinese Children's Leukemia Group (CCLG)-ALL 2008 (high risk group) protocol in the treatment with childhood Mixed phenotype acute leukemia (MPAL). METHODS: The clinical data of 15 new diagnosed patients with MPAL treated in our hospital from January 2013 to December 2017 were retrospectively analyzed, and received CCLG-ALL 2008 (high risk group) protocol chemotherapy. RESULTS: One patient gave up treatment after diagnosed, and 14 children with MPAL after induction remission chemotherapy, 3 patients gave up, and 5 patients received consolidation chemotherapy, and 6 patients received allogeneic hematopoietic stem cell transplantation (allo-HSCT). The complete remission (CR) rate was 85.7% at d33 of induction remission chemotherapy. The serious adverse event and treatment-related mortality (TRM) rate was 71.4% and 14.3%, respectively. The recurrence rate was 21.4% and the median time of relapse was 12(9.7-18.4) months. Except for 4 patients who gave up treatment, the 5-year event-free survival (EFS) rate in the other 11 patients was (54.5±15.0)%. The 5 years EFS of 4 patients who received consolidation chemotherapy was significantly lower than the 6 patients who received allo-HSCT after CR (25.0%±21.7% vs 83.3%±15.2%, P=0.033). CONCLUSION The CCLG-ALL2008 (for high-risk group) protocol in treatment of children with MPAL can get a high CR rate, but also with a high incidence of SAE. The patients received allo-HSCT after CR may have a good prognosis.
Child ; Disease-Free Survival ; Hematopoietic Stem Cell Transplantation ; Humans ; Leukemia, Myeloid, Acute ; Phenotype ; Prognosis ; Remission Induction ; Retrospective Studies

OBJECTIVE: To investigate the clinical features and prognostic factors of acute lymphoblastic leukemia (ALL) children with P2RY8-CRLF2 gene rearrangement. METHODS: A total of 108 children with B-cell ALL (B-ALL) were diagnosed and systematically treated according to Chinese Children's Leukemia Group (CCLG) -ALL 2008 in our hospital from January 2016 to December 2016. The 108 patients were divided into two groups according to the result of mutiplex polymerase chain reaction: group with P2RY8-CRLF2 gene rearrangement and group without P2RY8-CRLF2 gene rearrangement. The ALL children with P2RY8-CRLF2 gene rearrangement were all treated by CCLG-ALL 2008 high-risk group (HR) regimens, and the ALL children in group without P2RY8-CRLF2 gene rearrangement received different intensity chemotherapy according to clinical risk classification. RESULTS: Five (4 male and 1 female) out of 108 patients with B-ALL had P2RY8-CRLF2 gene rearrangement. In the 5 B-ALL patients with P2RY8-CRLF2 gene rearrangement, the median age of the was 4 (2-6) years old and the median WBC count was 26.2 (2.46-525.1)×10 CONCLUSION The early treatment response and prognosis of ALL children with P2RY8-CRLF2 gene rearrangement are worse, and more effective protocol is needed for this subtype patients.
Child ; Child, Preschool ; Disease-Free Survival ; Female ; Gene Rearrangement ; Humans ; Male ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/genetics* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics* ; Prognosis ; Receptors, Cytokine/genetics* ; Receptors, Purinergic P2Y/genetics*

OBJECTIVE: To analyze the expression of lncRNA-MALAT1 in peripheral blood of patients with acute myeloid leukemia (AML) sepsis and explore its clinical significance. METHODS: From March 2018 to March 2019, 95 confirmed AML patients including 43 sepsis infected cases and 52 uninfected cases were selected for treatment in the Department of Oncology and Hematology, The First People's Hospital of Longquanyi District. Their peripheral blood samples were taken as study samples, and the blood samples from 50 healthy people were used as control. RT-qPCR was used to detect lncRNA-MALAT1 expression level in samples from healthy group, uninfected group, and sepsis group. The correlation between lncRNA-MALAT1 expression level and clinical characteristics and prognosis of AML patients with sepsis were analyzed. RESULTS: The expression level of lncRNA-MALAT1 in the sepsis group was significantly up-regulated compared with the healthy group and uninfected group (P<0.05), while there was no significant difference between the healthy group and uninfected group (P>0.05). In AML patients with sepsis, the expression of lncRNA-MALAT1 was associated with clinical characteristics such as NCCN risk classification, white blood cell count, hemoglobin and so on. The overall survival rate of high lncRNA-MALAT1 expression group was significantly lower than that of low expression group (χ CONCLUSION The up-regulated expression of lncRNA-MALAT1 is closely related to the clinical characteristics and survival rate, and is an independent prognostic factor for AML sepsis patients. LncRNA-MALAT1 is expected to become a new diagnostic marker and therapeutic target for AML sepsis.
Humans ; Leukemia, Myeloid, Acute ; Prognosis ; RNA, Long Noncoding/genetics* ; Sepsis ; Survival Rate

OBJECTIVE: To explore the predictive value of newly diagnosed IgG levels in the recurrence of IgG-type multiple myeloma (MM) patients after initial treatment. METHODS: The clinical and pathological data of 91 patients newly diagnosed IgG-type MM who were hospitalized in the Department of Hematology of the Second People's Hospital of Yichang and Department of Oncology of The Affiliated Hospital of Jianghan University from April 2010 to March 2019 were collected. According to the median IgG level at the time of initial diagnosis, patients were divided into high IgG group and low IgG group. The recurrence time after initial treatment was followed up, and the correlation between newly diagnosed IgG level and recurrence was analyzed by univariate and multivariate analysis, as well as the influencing factors of IgG levels in order to predict furtherly the potential mechanism of recurrence. RESULTS: Univariate survival analysis showed that high revised international staging system (R-ISS) staging, high level of bone marrow plasma cell (BMPC), lactate dehydrogenase (LDH), creatinine, β CONCLUSION The higher the serum IgG concentration of IgG-type MM patients at first diagnosis, the earlier the recurrence, which is related to the low level of serum albumin, and can be used as a potential recurrence predictor after complete remission of IgG-type MM patients.
Bone Marrow ; Hematopoietic Stem Cell Transplantation ; Humans ; Immunoglobulin G ; Multiple Myeloma/therapy* ; Transplantation, Autologous

The mammalian target of rapamycin (mTOR)-sterol regulatory element-binding proteins (SREBPs) signaling promotes lipogenesis. However, mTOR inhibitors also displayed a significant side effect of hyperlipidemia. Thus, it is essential to develop mTOR-specific inhibitors to inhibit lipogenesis. Here, we screened the endogenous inhibitors of mTOR, and identified that FKBP38 as a vital regulator of lipid metabolism. FKBP38 decreased the lipid content

OBJECTIVE: To investigate the clinical features and prognostic factors of childhood Burkitt Lymphoma/leukemia. METHODS: The clinical data of 35 patients with newly-diagnosed childhood Burkitt lymphoma/leukemia from March 2011 to September 2017 in Fujian Medical University Union Hospital were retrospectively analyzed and summarized. Among 35 patients, 5 gave up treatment and one patient died of multiple organ failure before treatment, and 29 patients received CCCG-BNHL-2010 protocol chemotherapy. RESULTS: The 35 cases of BL/L includsd 31 males and 4 females (M∶F=7.75∶1) with the median age of 5(2.0-11) years. Clinically, the common infiltration sites were as follows: abdominal organs (especially ileocecus, 21/35, 60%), bone marrow (21/35, 60%), faciomaxillary (10/35, 28.57%), and central nervous system (8/35, 22.85%). According to St. Jude staging system, 6 patients were grouped into stage Ⅱ, and 8 into stage Ⅲ and 21 into stage Ⅳ, among which the bone marrow blasts of 17 patients were more than 25%. The analysis of therapeutic efficacy and prognosis showed that in median follow up of 23.4 (5.3-86.4) months, 5 patients relapsed (5/29, 17.24%), the median relapsed time was 5.7 (3.9-7.2) months; tow-year overall survival (OS) rate and progression-free survival (PFS) rate was 79.2%±7.6% and 78.3%±7.9%, respectively. Univariate analysis showed that the 2-year OS and PFS in patients with LDH＞2N, stage Ⅳ (bone marrow infiltration), central nervous system infiltration and no-CR after 2 courses of treatnent all were significantly lower than those in patients with LDH≤2N, stageⅡ-Ⅲ, without central nervous system infiltration as well as CR after 2 course of treatment (P values were 0.015, 0.015, 0.019 and 0.000, respectively). Cox regression analysis showed that no-CR after 2 course was an independent unfavorable prognostic factor (HR 0.34, 95%CI: 0.03-0.407). CONCLUSION The childhood Buruitts lymphoma/leukemia is more freguently seen in males and school-age children, Advanced stage, bone marrow and contral nervous system infitration are common at the first visit to doctor, moreover the Burkitt's lymphoma/leykemia present repid progression and dangerous feature. The current intensive chemotherapy (high dose of drugs and short course) possess the significant therapeutic efficacy for this disease, but the patients should have very poor prognosis if they can not achieve CR after 2 course of chemotherapy.
Antineoplastic Combined Chemotherapy Protocols ; Burkitt Lymphoma ; Child ; Child, Preschool ; Female ; Humans ; Male ; Prognosis ; Retrospective Studies

OBJECTIVE: To analyze the clinical features and to explore the therapeutic efficacy and prognostic factors of children with anaplastic large cell lymphoma (ALCL). METHODS: The clinical data of 18 children with ALCL admitted in Department of Pediatric Hematology, Union Hospital of Fujian Medical University from April 2011 to November 2017 was collected and analyzed. RESULTS: The male to female ratio was 2∶1, the median age of onset was 6 (0.9-11.3) years old, and the B symptom was positive in 13 cases. The most common initial symptom was lymphadenopathy (in 17 cases). All patients were manifested with multiple organ involvements. 4 cases were classified as clinical stage Ⅱ, 11 cases as stage Ⅲ, and 3 cases as stage Ⅳ. Laboratory tests revealed 9 cases with leukocytosis and 8 cases with CRP＞20 mg/L. The pathological results showed all ALK-positive anaplastic large cell lymphoma with Ki-67 rate between 40%-90%. The median follow-up time was 41 months. 2 patients died before treatment, 1 patient was lost to follow-up. 15 patients accepted chemotherapy protocol of CCCG-BNHL-2011. 2 patients relapsed early, the 3 year event-free survival rate was (76.7±10.2)%. Kaplan-Meier survival analysis showed leukocytosis, increased CRP level, bone involvement and clinical stage were factors affecting prognosis. CONCLUSION ALCL is a relatively rare subtype of childhood non-Hodgkin's lymphoma with high invasiveness. Leukocytosis, increased CRP level, bone involvement and clinical stage are poor factors affecting the prognosis of patients.
Antineoplastic Combined Chemotherapy Protocols ; Child ; Child, Preschool ; Disease-Free Survival ; Female ; Humans ; Infant ; Lymphoma, Large-Cell, Anaplastic ; Male ; Prognosis