Objective:To evaluate the clinical characteristics, pathology, treatment and prognosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in children.Methods:Clinical data (including gender, age of disease onset, affected sites, treatment, timing of allogeneic hematopoietic stem cell transplantation (allo-HSCT), etc.) of 7 children with BPDCN who were admitted to Beijing Children′s Hospital, Capital Medical University from December 2018 to December 2023 were analyzed retrospectively. Clinical outcomes were also assessed, with patients followed up until December 2024.Results:Among 7 patients, there were 3 males and 4 females. Age at disease onset ranged from 3.2 to 12.9 years. Initial presentations included subcutaneous nodules in 5 cases, rash in 1 case, and ankle pain in 1 case. Extra-cutaneous involvement was seen in the bone marrow, lymph nodes, and central nervous system. Six patients received induction chemotherapy using a modified lymphoblastic lymphoma regimen, 1 patient received the high-risk protocol for pediatric lymphoblastic lymphoma/leukemia and salvage therapy regimens. Allo-HSCT was performed soon after chemotherapy remission. The time to bridge allo-HSCT was 3.5 to 6.5 months. The follow-up time was 1.6 to 6.0 years. Six patients were in disease-free survival, while 1 patient survived with disease after recurrence following transplantation.Conclusions:BPDCN is rare in children and presents diverse clinical manifestations, with skin involvement being the predominant feature. Early allo-HSCT following complete remission with chemotherapy can improve prognosis.

Objective:To evaluate the clinical characteristics, pathology, treatment and prognosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in children.Methods:Clinical data (including gender, age of disease onset, affected sites, treatment, timing of allogeneic hematopoietic stem cell transplantation (allo-HSCT), etc.) of 7 children with BPDCN who were admitted to Beijing Children′s Hospital, Capital Medical University from December 2018 to December 2023 were analyzed retrospectively. Clinical outcomes were also assessed, with patients followed up until December 2024.Results:Among 7 patients, there were 3 males and 4 females. Age at disease onset ranged from 3.2 to 12.9 years. Initial presentations included subcutaneous nodules in 5 cases, rash in 1 case, and ankle pain in 1 case. Extra-cutaneous involvement was seen in the bone marrow, lymph nodes, and central nervous system. Six patients received induction chemotherapy using a modified lymphoblastic lymphoma regimen, 1 patient received the high-risk protocol for pediatric lymphoblastic lymphoma/leukemia and salvage therapy regimens. Allo-HSCT was performed soon after chemotherapy remission. The time to bridge allo-HSCT was 3.5 to 6.5 months. The follow-up time was 1.6 to 6.0 years. Six patients were in disease-free survival, while 1 patient survived with disease after recurrence following transplantation.Conclusions:BPDCN is rare in children and presents diverse clinical manifestations, with skin involvement being the predominant feature. Early allo-HSCT following complete remission with chemotherapy can improve prognosis.

Objective:To evaluate the efficacy and safety of brentuximab vedotin (BV) combined with rituximab and attenuated chemotherapy in the treatment of children with classic Hodgkin lymphoma (cHL).Methods:A prospective, non-randomized, risk-assigned study. Clinical data (including age, gender, B symptoms, bulky disease, CD30 and Epstein-Barr virus-encoded RNA(EBER) expression, clinical stage, risk stratification, etc.) of 28 intermediate to high-risk cHL children diagnosed and treated at Beijing Children′s Hospital Affiliated to Capital Medical University from October 2022 to May 2024 were collected. Immuno-targeted combined with attenuated chemotherapy was administered based on risk stratification and early treatment response. The patients were followed up until May 1st, 2024. The infusion reactions and adverse reactions after treatment were recorded.Results:In all 28 patients, there were 22 males and 6 females, the age was 12 (5,16) years, 16 cases (57%) presented with bulky disease and 10 cases (36%) with B symptoms. The most common pathological type was nodular sclerosis (14 cases, 50%). There were 7 cases of stage Ⅱ, 14 cases of stage Ⅲ and 7 cases of stage Ⅳ according to the Ann Arbor staging system. There were 5 cases in the intermediate-risk group and 23 cases in the high-risk group. EBER was positive in 20 cases (71%) and negative in 6 cases (21%), and CD30 antigen was expressed in tumor cells of all enrolled children. Treatment duration: 5 cases (18%) received 4 courses of treatment, 21 cases (75%) received 6 courses of treatment, and 2 cases (7%) received 8 courses of treatment, 25 cases (89%) achieved complete metabolism response (CMR) through early assessment after 2 courses of chemotherapy. The CMR rates were 100% in intermediate-risk group and 87% (20/23) in high-risk group, respectively. Four patients (14%) finally received residual field radiotherapy. Toxicities included grade Ⅰ-Ⅱ myelosuppression, early infusion reaction and mild peripheral neuropathy, only one case of grade 3 adverse events was recorded and did not affect sequential treatment. At the end of treatment and 3 months of follow-up, the levels of IgA, IgG and IgM were all decreased compared with the baseline before chemotherapy, and the total B cell count began to be lower than the level before chemotherapy at the early stage of treatment (after 2 courses). The total B cell count monitored during treatment was 50 (0, 101)×10 6/L and was 12 (0, 25)×10 6/L at the end of treatment. The follow-up time was 6 (3, 13) months, all 28 children had event-free survival and all achieved complete remission. At 6 and 9 months of follow-up, IgA, IgG, IgM and total B cell counts returned to pre-chemotherapy baseline levels, respectively. Conclusion:BV combined with rituximab attenuated chemotherapy has demonstrated efficacy and a tolerable safety profile in the treatment of cHL in children, and significantly reduce radiation rate.

Clinical data of 15 primary central nervous system lymphoma (PCNSL) children aged ≤18 years admitted to our hospital between May 2013 to May 2023 were retrospectively analyzed. Our goal was to summarize the clinical features of children and investigate the therapeutic effect of a high-dose methotrexate (HD-MTX) based chemotherapy regimen on this disease. The male-to-female ratio was 2.7∶1, and the median age was 7.2 (2.3-16.4) years at diagnosis. The initial clinical symptoms were primarily cranial hypertension, with imaging findings revealing multiple lesions. Pediatric PCNSL with normal immune function has a favorable prognosis with HD-MTX-based chemotherapy. Patients with a stable disease can be treated with minimal or no maintenance. HD-MTX-based chemotherapy remains effective when the disease progresses or recurs after an initial course of non-HD-MTX-based chemotherapy.

Objective:To investigate the clinical manifestations, pathological features, treatment and prognosis of primary bone lymphoma in children.Methods:The clinical data of children who were initially diagnosed as primary bone lymphoma and treated in Beijing Children's Hospital Affiliated to Capital Medical University from January 2016 to January 2020 were retrospectively analyzed, including gender, onset age, primary involvement site, clinical stage, pathological type, fracture, and clinical outcome. The related literature was reviewed.Results:All 11 children were initially diagnosed as primary bone lymphoma, with a median age of onset of 8.6 years old (2.7-12.3 years old), including 7 males and 4 females. There were 7 cases of diffuse large B-cell lymphoma (DLBCL), 3 cases of B lymphoblastic lymphoma (BLL), and 1 case of anaplastic large cell lymphoma (ALCL). The initial symptoms were bone pain in 8 cases, local swelling in 1 case, limp in 1 case, and fever in 1 case. One case was in stage Ⅰ, 7 cases were in stageⅡ, and 3 cases were in stage Ⅳ, and the most common sites of involvement were femur and tibia. All 11 cases were treated with chemotherapy according to different pathological types, with a median follow-up time of 45 months (7-80 months). Ten cases got complete remission, 1 case of BLL died of bone marrow recurrence after chemotherapy remission.Conclusions:The clinical manifestations of primary bone lymphoma in children are insidious, DLBCL is the most common pathological type, and the prognosis is good after standardized treatment.

A male child, aged 5 years and 3 months, was admitted to the Oncology Department with a history of pain in both hip joints, headache, and diplopia lasting for 40 days. Physical examination did not reveal definitive signs or obvious abnormalities in the nervous system. Imaging studies showed only abnormalities in the craniocerebrum and spinal cord. Routine cerebrospinal fluid (CSF) analysis revealed elevation in the total number of white blood cells, mainly mononuclear cells. Biochemical analysis of CSF showed normal glucose and chloride levels, and increased protein concentrations. The possibility of central nervous system (CNS) infection was initially considered. Subsequently, antibacterial and antiviral therapy was administered; however, this treatment was ineffective. Further examination of CSF through immunophenotyping revealed mature B-cell lymphoma with CNS involvement; there were no neoplastic lesions detected elsewhere in the body. Thus, the patient was diagnosed with primary central nervous system lymphoma (PCNSL). Complete remission was achieved after chemotherapy with the CNCL-2017-mature B-cell lymphoma regimen. Thus far, all chemotherapy cycles have been completed, the patient remains in complete remission, and the follow-up is ongoing. Clinicians should pay close attention to PCNSL in children.

Objective:To investigate the clinical characteristics of pregnant women with leukemia, the condition of leukemia and the influence of clinical treatment on maternal and infant outcomes, and to explore the best clinical management method of leukemia in pregnancy.Methods:Among 79 890 pregnant and lying-in women in Qilu Hospital of Shandong University from January 2004 to December 2015, 22 cases (0.028%) were with leukemia, including 5 cases of leukemia diagnosed before pregnancy [all acute myeloid leukemia (AML)] and 17 cases of leukemia diagnosed for the first time after pregnancy [9 cases of AML, 5 cases of chronic myeloid leukemia (CML), 2 cases of acute lymphoblastic leukemia (ALL), and 1 case of chronic lymphocytic leukemia (CLL)]. According to the gestational weeks of admission and confirmed gestational weeks of leukemia, the 22 patients were divided into early-stage group (initial gestational week < 14 weeks, 5 cases), mid-stage group (newly diagnosed gestational week ≥ 14 weeks and < 28 weeks, 11 cases), and late-stage group (newly diagnosed gestational week ≥ 28 weeks, 6 cases, including 2 cases with previous diagnosis of leukemia). The final pregnancy outcomes included abortion, induced labor, premature delivery, full-term delivery and maternal and infant death. The effects of clinical treatment and obstetric treatment of leukemia on the final maternal and infant outcomes, follow-up to understand the progress of primary disease and fertility of pregnant women, and the impact of leukemia and pregnancy treatment on long-term health status of infants were analyzed.Results:Among 22 patients with leukemia in pregnancy, 14 cases (63.6%) (5 cases in early-stage group and 9 cases in mid-stage group) choosed to give up pregnancy, including 4 cases of early pregnancy abortion and 10 cases of mid pregnancy induced abortion; 12 cases of 14 cases were induced abortion or induced labor after leukemia remission induced by advanced chemotherapy. The remaining 8 patients (2 cases in mid-stage group and 6 cases in late-stage group) continued pregnancy and gave birth to live infants, of which 3 cases received chemotherapy before delivery.Conclusions:Gestational leukemia is a high-risk obstetric case, but it is still expected to achieve good pregnancy outcome under good management and treatment. On the basis of following the principles of leukemia treatment, according to the gestational weeks and patients' wishes, the individualized clinical management plan is formulated, and the accurate chemotherapy timing is conducive to the prognosis of mother and infant.

Objective To investigate the significant of peripheral CD4+CD69+T lymphocytes in patients with autoimmune hemolytic anemia (AIHA)/Evans syndrome (ES).Methods In this study peripheral blood samples from 32 patients with AIHA/ES (15 hemolytic episode patients,17 remission patients) and 13 healthy controls were collected.Patients with AIHA/ES were recruited in Tianjin Medical University General Hospital from October 2015 to May 2016.The percentages of CD69+ T lymphocytes were analyzed by flow cytometry.The expression of CD69 mRNA in CD4+T lymphocytes which was sorted from peripheral blood by magnetic activated cell sorting (MACS) was detected using real-time PCR.Soluable CD69 was measured by ELISA.Results In hemolytic episode patients,the ratio of CD3+CD69+/CD3+T lymphocytes [(3.08 ± 1.48)％] was significantly higher than that in healthy controls [(1.28 ± 0.83)％,P＜0.01] and in remission group[(1.96± 1.33)％,P＜0.05].The absolute count of CD3+CD69+T lymphocytes in hemolytic episode group [(2.94± 1.81)× 107/L] was higher than that in healthy controls [(1.48± 1.42)× 107/L,P＜0.05].The ratio of CD3+CD4+CD69+/CD3+CD4+T cells in hemolytic episode group [(2.16± 1.56)％] was significantly higher than that in remission group [(1.16±0.62)％,P＜0.05] and healthy controls[(0.94±0.78)％,P＜0.05].The quantity of CD3+CD4+CD69+T lymphocytes in hemolytic episode group[(1.04±0.98)× 107/L] was higher than in healthy controls [(0.44± 0.38) × 107 / L,P＜0.05].The ratio of CD3+CD8+CD69+/CD3+ CD8+T lymphocyte in hemolytic episode group [(4.87±2.56)％] was significantly higher than that in healthy controls[(1.83± 1.27)％,P＜0.01].The quantity of CD3+CDs+CD69+T lymphocytes in three groups did not show significant difference.The ratio of CD3+CD4+CD69+/CD3+CD4+T lymphocytes in hemolytic episode group was negatively correlated with hemoglobin (Hb) (P＜0.01),positively correlated with the percentage of reticulocytes (Ret％)(P=0.01)total bilirubin(TBil),indirect bilirubin(IBil) (P＜0.01) and not correlated with absolute reticulocytes count,lactic dehydrogenase (LDH),complement 3(C3),complement 4 (C4).The ratio of CD3+CD4+CD69+/CD3+CD4+T lymphocytes in remission group was negatively correlated with Hb (P＜0.05).In hemolytic episode patients CD69 mRNA (32.26±35.11) was significantly higher than that in remission group(6.05±5.87)(P＜0.05)and healthy controls (1.76± 1.85)(P＜0.01).CD69 mRNA in remission group was significantly higher than healthy controls (P＜0.05).Serum CD69 in hemolytic episode patients [(494.21 ± 16.06) ng/L] was significantly higher than that in healthy controls [(441.39± 104.6) ng/L,P＜0.05].Conclusion Our findings suggest that the proportion of CD4+CD69+ T lymphocytes increase in AIHA/ES patients,which is correlated with the severity of disease.

Objective The objective of this study was to investigate the miR-345 expression level in non-small cell lung cancer(NSCLC)tissue and its potential clinical significance.Methods Real-time PCR was conducted to evaluate the expression of miR-345 in NSCLC tissues in 98 samples and cell lines.Then the association between tissue miR-345 expression level and clinical outcomes was further analyzed.Results The expression level of miR-345 was significantly decreased in NSCLC tissues and cell lines when compared to the controls(P<0.05).miR-345 expression level in NSCLS tissues was associated with various clinicopathological parameters including LN metastasis(P<0.001),distant metastasis(P=0.028),TNM stage(P=0.004)and grade(P=0.011).In addition,the NSCLC patients in the low miR-345 expression group showed significantly shorter overall survival time than that in the high miR-345expression group(P=0.021).Multivariate analysis showed that the expression of miR-345 was an independent risk factor for NSCLC(HR=3.897,95% CI:2.263~10.440;P=0.012).Conclusion The expression level of miR-345 was decreased in NSCLC tissues as well as cell lines compared with normal controls.Low miR-345 expression in tissues was associated with progression and poor prognosis of NSCLC.These results indicate that miR-345 may be a novel prognostic marker in NSCLC.

Objective To observe the effect of continuous incision infusion different concentra-tion of ropivacaine for postoperative analgesia after radical mastectomy.Methods One hundred pa-tients under radical mastectomy,aged 40-70 years,ASA Ⅰ or Ⅱ,were randomly divided into four groups (n =25 each):0.2% (group R1),0.3% (group R2),0.4% (group R3)ropivacaine incision continued infiltration group and patient-controlled intravenous analgesia (group PCIA)as control group.VAS pain scores,sedation Ramsay score and side effects were recorded at each time point in rest and turning over 90°,2 h (T1 ),4 h (T2 ),8 h (T3 ),12 h (T4 ),24 h (T5 ),48 h (T6 )after the operation.Results VAS scores in group R1 at T1-T6 in rest and turn over 90°were significantly high-er than that of group PCIA (P <0.05).There were no significant differences among the group PCIA, group R2 and group R3.Sedation score in PCIA group was significantly higher than that in the other three groups (P <0.05),and the adverse reactions,such as nausea and vomiting,in group PCIA (2 cases)were more serious than that in the other groups (0 cases ).There were no significant differences among the other groups.Conclusion Ropivacaine plays an effective role in infiltration an-algesia when its concentration reaches 0.3% subcutaneous after radical mastectomy.