Objective:To investigate the effects of hemophagocytic syndrome also known as hemophagocytic lymphohistiocytosis (HLH) on the clinical features and therapeutic efficacy of patients with Epstein-Barr virus-positive T-cell lymphoma (EBV-TCL).Methods:The clinical data of patients with EBV-TCL diagnosed by pathological examination in the First Affiliated Hospital of Guangzhou Medical University from November 2015 to August 2020 were retrospectively analyzed. According to whether they were accompanied with HLH at the time of onset, patients were divided into HLH group (10 cases) and non-HLH group (13 cases), and the clinical features and prognosis of the two groups were compared. The curative effects of different treatment methods and patients with different plasma EBV-DNA titers were compared.Results:Among 23 patients, 3 cases (13.0%) were in Ann Arbor stage Ⅰ-Ⅱ, 20 cases (87.0%) were in stage Ⅲ-Ⅳ; the International Prognostic Index (IPI) score was 1 point in 3 cases (13.0%), 2 points in 4 cases (17.4%), 3 points in 8 cases (34.8%), 4 points in 8 cases (34.8%). In the HLH group, there were 2 cases of aggressive NK-cell leukemia and 3 cases of childhood systemic EBV-TCL. There were no cases of above two pathological types in the non-HLH group. In the HLH group, the proportions of patients with fever, bone marrow invasion, IPI score > 2 points, and EBV-DNA > 10 4 copies/ml were higher than those in the non-HLH group (all P < 0.05). The objective response rate (complete remission plus partial remission) of all patients after chemotherapy was 47.8% (11/23); there were 3 cases undergoing hematopoietic stem cell transplantation in both the HLH group and the non-HLH group, and all achieved objective remission. The objective remission of 7 patients and 10 patients who did not undergo hematopoietic stem cell transplantation in the HLH group and non-HLH group after lymphoma chemotherapy had 0 case and 5 cases, respectively, and the difference was statistically significant ( P = 0.044). In the chemotherapy alone group, 5 of 17 patients had objective remission, 6 patients in the chemotherapy plus transplantation group had objective remission, and the difference was statistically significant ( P = 0.039). Among 16 patients whose plasma EBV-DNA titers turned negative, 11 patients had objective remission, and 7 patients whose plasma EBV-DNA titers were continuously positive had no objective remission, and the difference was statistically significant ( P = 0.001). The 1-year overall survival rate of all patients was 69.3%, and the 2-year overall survival rate was 52.0%. In the HLH group, the 1-year and 2-year overall survival rates of 7 patients receiving chemotherapy alone and 3 patients receiving chemotherapy plus transplantation were 42.9% and 66.7%, respectively. In the non-HLH group, the 1-year overall survival rates of 10 patients receiving chemotherapy alone and 3 patients receiving chemotherapy plus transplantation were 80.0% and 100.0%, respectively; the 2-year overall survival rates were 26.7% and 100.0%,respectively. The overall survival of patients receiving chemotherapy plus transplantation was better than that of those receiving chemotherapy alone in both the HLH group and the non-HLH group, and differences were statistically significant (all P < 0.05). Conclusions:The general clinical stage of patients with EBV-TCL is later, and the prognosis of EBV-TCL patients with HLH is worse. The therapeutic efficacy may be related to plasma EBV-DNA titers. Hematopoietic stem cell transplantation can improve the remission rate.

Objective:To invetigate the influencing factors and clinical significance of liver function damage (LFD) in patients diagnosed with Corona Virus Disease 2019 (COVID-19).Methods:The retrospective case-control study was conducted. The clinicopathological data of 51 patients with COVID-19 who were admitted to the Sino-French New City Branch of Tongji Hospital Affiliated to Huazhong University of Science and Technology by the 5th group assisting team from the First Hospital of Jilin University from February 9th to 27th in 2020 were collected. There were 27 males and 24 females, aged from 36 to 86 years, with an average age of 68 years. The treatment modality was according to the diagnostic and therapeutic guideline for COVID-19 (Trial 6th edition) issued by National Health Commission. Observation indicators: (1) clinical data of patients; (2) analysis of liver function index and treatment of LFD; (3) analysis of influencing factors for LFD. Measurement data with normal distribution were represented as Mean± SD, and measurement data with skewed distribution were described as M (range). Count data were described as absolute numbers or percentages, and comparison between groups was analyzed using the chi-square test. The Logistic regression method was used for univariate analysis. Results:(1) Clinical data of patients: of the 51 patients, 21 were classified as ordinary type of COVID-19, 19 as severe type and 11 as critical type. In terms of medical history, 31 patients suffered from more than or equal to one kind of chronic disease, 20 had no history of chronic disease. Thirteen patients had the drinking history and 38 had no drinking history. Seven patients were hepatitis positive and 44 were hepatitis negative. Five patients had septic shock at admission, 5 had systemic inflammatory response syndrome (SIRS), and 41 had neither shock nor SIRS. The body mass index (BMI), time from onset to admission, temperature, heart rate, respiratory rate of the 51 patients were (24±3)kg/m 2, (13±5)days, 36.5 ℃ (range, 36.0-38.1 ℃), 82 times/minutes (range, 50-133 times/minutes), 20 times/minutes (range, 12-40 times/minutes). The white blood cell count, level of creatinine, and level of b-type natriuretic peptide within 24 hours after admission were 6.3×10 9/L [range, (2.2-21.7)×10 9/L], 75 μmol/L (range, 44-342 μmol/L), 214 ng/L (range, 5-32 407 ng/L). (2) Analysis of liver function index and treatment of LFD: the level of alanine aminotransferase (ALT), aspartate aminotransferase (AST), glutamyl transpeptidase (GGT), alkaline phosphatase (ALP), direct bilirubin (DBil), indirect bilirubin (IBil), activated partial thromboplastin time (APTT) and prothrombin time (PT) were 31 U/L (range, 7-421 U/L), 29 U/L (range, 15-783 U/L), 36 U/L (range, 13-936 U/L), 76 U/L (range, 41-321 U/L), 4.9 μmol/L (range, 2.6-14.3 μmol/L), 5.8 μmol/L (range, 2.6-23.9 μmol/L), 37.2 s (range, 30.9-77.1 s), 13.9 s (range, 12.5-26.7 s), respectively. The percentages of cases with abnormal ALT, AST, GGT, ALP, DBil, IBil, APTT and PT were 47.1%(24/51), 47.1%(24/51), 35.3%(18/51), 13.7%(7/51), 7.8%(4/51), 2.0%(1/51), 21.6%(11/51), and 19.6%(10/51), respectively. Of the 51 patients, LFD was detected in 10 patients classified as ordinary type, in 9 patients as severe type, and in 10 as critical type, respectively. In the 51 patients, 1 of 22 patients with normal liver function developed respiratory failure and received mechanical ventilation within 24 hours after admission, while 9 of 29 patients with abnormal liver function developed respiratory failure and received mechanical ventilation, showing a significant difference between the two groups ( χ2=5.57, P<0.05). (3) Analysis of influencing factors for LFD. Results of univariate analysis showed that clinical classification of COVID-19 as critical type was a related factor for LFD of patients ( odds ratio=10.000, 95% confidence interval: 1.050-95.231, P<0.05). Conclusions:COVID-19 patients with LFD are more susceptible to develop respiratory failure. The clinical classification of COVID-19 as critical type is a related factor for LFD of patients.

To analyze respectively a case,presented with recurrent petechial and epistaxis after a 2 years history of diabetes mellitus (DM),who was hospitalized in Beijing Children's Hospital Affiliated to Capital Medical University.The clinical manifestation,examination,diagnosis and treatment were recorded.The patient was diagnosed with immune thrombocytopenia (ITP) and DM at the first admission.The initial therapy with gamma globulin didn't show ideal effect.The pediatric specialists from the department of ENT,Hematology/Oncology,Endocrinology,Pharmacy and Immunodeficiency Clinic were invited to discuss the case.The final diagnosis of autoimmune polyglandular syndrome (APS) was made and supplementary steroid treatment was started.But the response of the steroid therapy was poor.Once again with the multidisciplinary consultation,the patient received several schemes of Rituximab under the informed consent.This treatment reached a stable condition for almost 7 years.APS should be considered when DM patient showed the manifestation of other immune organ damages.Rituximab immunosuppressive therapy should be tried when the response to first-line treatment was poor.

Objective: To evaluate the efficacy and safety of imatinib in the treatment of newly diagnosed chronic myeloid leukemia during chronic phase (CML-CP) in children and to analyze the difference of the efficacy and safety between imported original imatinib (Gleevec) and domestic generic imatinib (Xinwei). Methods: Clinical data of 35 children with newly diagnosed CML-CP in Beijing Children′s Hospital from January 2014 to January 2018 were collected, among which 15 cases were treated with the imported original imatinib (original drug group) and 20 cases were treated with the domestic generic imatinib (generic drug group). The hematological, cytogenetic and molecular reactions and safety of the treatments were monitored at months 3, 6 and 12. Chi square test or rank sum test was used for the comparison between two groups. Results: A total of 35 cases were treated for over 3 months, 31 cases were treated for over 6 months and 25 cases were treated for over 12 months. At 3 months, main cytogenetic response was obtained in 15 (100%) cases in the original drug group and 16 (80%) cases in the generic drug group respectively (χ²=3.387, P=0.119). At 6 months, complete cytogenetic response was obtained in 12 (80%) cases in the original drug group and 10 (63%) cases in the generic drug group (χ²=1.435, P=0.390). At 12 months, BCR-ABL^IS ≤ 0.1% was obtained in 11 (92%) cases in the original drug group and 10 (77%) cases in the generic drug group (χ²=1.009, P=0.593). There was no significant difference at all stages (all P>0.05). Hematologic toxicity occurred in 7(20%) cases. The non-hematologic adverse reactions include nausea in 8 (23%) cases, pain in 8 (23%) cases, edema in 6 (17%) cases, emesis in 2 (6%) cases, fever in 2 (6%) cases, weakness in 1 (3%) case, rash in 1 (3%) case. The adverse reactions were easy to control and no drug toxicity related deaths occurred. There was no significant difference in the adverse reactions between original drug group and generic drug group (P>0.05). Conclusions Imatinib had a good efficacy and safety in the early treatment of newly diagnosed CML-CP in children. The efficacy and safety of generic imatinib is similar to that of imported imatinib.

Objective To analyze the correlation between genotype,clinical manifestations and treatment response in patients diagnosed as Diamond-Blackfan anemia (DBA) with a clear pathogenic gene mutation.Methods Retrospective investigation was performed of the genetic and clinical data of 42 patients diagnosed as DBA with a definite mutation from December 2009 to October 2017 in Beijing Children's Hospital,Capital Medical University and DBA China group.Results Among 42 patients,no one patient could successfully stop the therapy during the median follow-up time of 40 months (1-136 months).Nucleotide-level mutations or large deletions were identified in 7 ribosomal genes as RPL5,RPL11,RPL35a,RPS17,RPS19,RPS24 and RPS26.The most common gene mutation group was RPS19 (42.9％),followed by RPL11(19.0％),RPS17(11.9％),RPS26(11.9％),RPL5 (7.1％) and RPL35a(4.8％).The median onset hemoglobin level was 42.5 g/L.A total of 12 patients had physical malformation,with the most common on heart and fingers.A total of 37 patients received hormone therapy,and the total initial response rate was 89.2％ (33/37 cases).One of the patients with hormone inefficiency was treated successfully with cyclosporin A,and the other 3 patients were treated with blood transfusion.Conclusions RPS19 was the most common gene mutation in DBApatients.Most of the RPS17 mutations were copy number variation.The deletion of large fragments should be paid more attention to in the detection of DBA genetic analysis.Patients with RPL5 mutation showed more malformation than other groups.No significant difference was found in terms of age of onset,hemoglobin level of onset,incidence of malformation and effective rate of hormone treatment in each group.

Objective To evaluate the impact of recombinant human thrombopoietin(rhTPO)in response of immunosuppressive therapy(IST)in children with severe aplastic anemia(SAA). Methods A retrospective analysis was made. The clinical data included 70 children with acquired aplastic anemia (AA)treatment with antithymocyte globulin(ATG)combined with Cyclosporine at Beijing Children's Hospital,Capital Medical University between January 2009 and December 2016. Thirty - six cases received standard IST(IST group),and 34 cases received standard IST combined with rhTPO (IST + rhTPO group ). The SPSS 19. 0 statistical package was used for data analysis. Results Three months after IST,the hematologic response rate in the IST + rhTPO group was much higher than that in the IST group [88. 2％(30 / 34 cases)vs. 33. 3％ (12 / 36 cases),and there was significant difference between 2 groups (χ2 = 21. 961,P = 0. 000). The hematologic response rate at 6 months,12 months and the endpoint of follow - up in the IST + rhTPO group were 76. 7％(23 / 30 cases),78. 9％(15 / 19 cases),70. 6％(24 / 34 cases),and which in the IST group were 63. 9％(23 / 36 cases),75. 1％(27 / 36 cases),75. 0％(27 / 36 cases),which were not significant difference between the 2 groups(all P > 0. 05). The median time of partial remission (PR)was 1. 5 months,and the median time of complete remission (CR)was 6 months in IST + rhTPO group. The median time of PR was 4 months,and the median time of CR was 14 months in the IST group. By the endpoint of follow - ups,the median time of platelet transfusion in-dependence(33 d)and red cell transfusion independence(39 d)in the rhTPO group were all shorter than those in the IST group(78. 0 d and 93. 5 d,respectively),and there were a significant differences between the 2 groups(all P <0. 05). By 3 months after IST,the level of platelet,hemoglobin and absolute reticulocyte counts(ARC)in the IST +rhTPO group were much higher than those in the IST group(48 × 109 / L vs.14 ×109 / L,104. 5 g/ L vs 66. 5 g/ L,60.34 × 109 / L vs. 50. 92 × 109 / L,respectively),and there were significant differences between the 2 groups (all P < 0. 05). There was no differences of those at 6 months,12 months after IST between the 2 groups(all P > 0. 05). The 3 - year overall survival(OS)rate was 100. 0％(34 / 34 cases)in the IST + rhTPO group,87. 5％(32 / 36 cases)in the IST group,and there was no significant difference between the 2 groups (P > 0. 05). Conclusions Three months of rhTPO treatment after IST could improve early hematologic response rate in children with newly diagnosed acquired SAA. It may speed up recovery of platelet and hemoglobin levels and had no significant impacts on long - term hematologic response rate,platelet or hemoglobin recovery and OS rate.

Objective: To investigate the current status of catheter-related-thrombosis (CRT) and the risk factors of Chinese acute lymphocytic leukemia (ALL) children with peripherally inserted central catheter (PICC) . Methods: The clinical data of the 116 inpatients preliminarily diagnosed ALL in the Leukemia Ward of Beijing Children’s Hospital with PICC from 1^st March 2014 to 31^st December 2014 were collected prospectively. Results: ①Refer to the B-ultrasound on the 15^th day after catheterization, the incidence of CRT was 28.4% (33/116 cases) , all cases were symptom-free. ②There were no statistical differences in terms of gender, age distribution, degree, immunotype between CRT and CRT-free groups. This study revealed no statistical differences of blood routine test items, coagulation function items, co-infection and catheterization vein between the two groups. While there was significant statistical difference of catheterization side, the frequency of right catheterization was higher in CRT group[75.8% (25/33) vs 55.4% (46/83) , P=0.043]. ③On the 15^th day after catheterization, significant statistical difference of D-Dimer between the two groups was revealed[0.18 (0.05-2.45) mg/L vs 0.11 (0.01-5.34) mg/L, P=0.001], while no statistical differences of blood routine test items and other coagulation function items. Multivariate Logistic regression analysis verified catheterization on right was a risk factor of CRT. ④During the observation, there were 3 cases of catheter-related complications other than CRT, all of which were CRI, 2 of them had CRT meanwhile. ⑤The B-ultrasound on the 33^rd day after catheterization showed that 73.1% of the cases had reduced thrombosis, 3.8% had growth thrombosis, 23.1% had no obvious change respectively. Conclusion CRT was a common catheter related complication among ALL children during induction chemotherapy, and CRT cases with symptoms were rare. Catheterization on right was a risk factor for CRT, and regular test of D-Dimer and B ultrasound contributed to detect CRT. Most of the CRT cases had reduced thrombosis without specific management.

Objective To study the current status of catheter - related thrombosis(CRT)in Chinese children through a retrospective analysis of the inpatients in the Department of Medicine,Beijing Children's Hospital Affiliated to Capital Medical University. Methods The clinical data of the inpatients with CRT from November 2010 to November 2013 were collected retrospectively,and the causes,clinical symptoms,diagnosis,treatment and prognosis were ana-lyzed. Results There were 42 cases of children with CRT in Beijing Children's Hospital Affiliated to Capital Medical University. Among the cases,the male to female ratio was 1. 0:0. 5;the median age of onset was 88(2 - 186)months with ﹤ 1 year old counted for 16. 7%(7 / 42 cases)and 13 - 14 years old counted for 11. 9%(5 / 42 cases);the distri-bution differences between the male and the female age were not significant(P = 0. 826). The median time from cathe-terization to CRT onset was 9(1 - 81)days,0 - 10 days after catheterization was the peak of onset(52. 5% ,21 / 40 ca-ses)followed by 10 - 20 days(35. 0% ,14 / 40 cases). The protopathy was usually hematologic tumor,kidney disease or deep fungal infection. Slightly more cases developed CRT on the right side(57. 1% ,24 / 42 cases)than on the left side (38. 1% ,18 / 42 cases). All cases were diagnosed by using B - ultrasound,of whom 28. 6%(12 / 42 cases)were symp-tom - free. After being diagnosed,7. 1%(3 / 42 cases)were treated with conservative methods such as immobilization of the affected limbs and hot compress;7. 1%(3 / 42 cases)had catheter removed;anticoagulant and/ or thrombolytics after catheter removal used in 33. 3% patients(14 / 42 cases). After 1 week,22 cases were reviewed,of whom 54. 5%(12 / 22 cases)had thrombosis reduced(all with intervention),thrombosis growing in 22. 7% patients(5 / 22 cases), and thrombosis did not change in 22. 7% patients(5 / 22 cases). Three cases needed re - catheterization after catheter removal,and all of 3 cases had CRT recurrences(100% ). Conclusions CRT is more common among infants and senior children. CRT usually develops within 20 days after catheterization. Children with hematologic tumor,kidney disease or deep fungal infection are more likely to have CRT. Routine ultrasound test should be conducted to monitor CRT in catheterized children. Once CRT is diagnosed,patients need to be treated with anticoagulants and/ or thrombo-lytics. Catheter should also be removed if necessary. Recatheterization can result in CRT recurrence.

Objective To collect and summarize long-term complications of glucocorticoid (GC) in treatment of Diamond-Blackfan anemia (DBA).Methods A total of 17 DBA patients,treated with GC more than 1 year from December 2009 to November 2012 in Being Children's Hospital,Capital Medical University,were retrospectively investigated.The data of general information,drug treatment,treatment response,height and body mass in different therapy periods,and therapy related adverse reaction were collected.The data entry and the statistical analysis were performed using SPSS 16.0 software.Results Seventeen cases which fulfilled the research criteria were enrolled.The 58.8 percent of cases (10/17) began GC therapy from the age younger than 6 months.The 76.4 percent of the cases (14/17) started prednisone therapy with the dosage ≥2 mg/(kg · d),and the median time of maintenance therapy with this dosage was 2 months (1-5 months).The median time of prednisone dosage greater than 0.5 mg/(kg · d) was 6 months (3-48 months).Patients were divided into 2 groups at the beginning of therapy according to whether their age was younger or older than 6 months.The median height of younger age group was-1.0 SD (-3.5-1.0 SD) of corresponded age-sex-standard height at the beginning of prednisone therapy,and was dropped to-3.5 SD (-3.5--2.0SD)afterhalfyeartreatment.For older age group,it was0.0 SD(-1.5-2.0 SD)and-0.5 SD (-1.5-0.5 SD) respectively.During the therapy,there were 1 fracture,2 measles pneumonia,3 pneumonia,3 hirsuitisms,5 thrushes,and 12 central obesity cases.Conclusions GC related adverse reaction might appear when applying prednisone for the treatment of DBA in long term.It was suggested that GC therapy should start after 6 months old if possible,and the duration of 0.5 mg/(kg · d) GC treatment should be reduced as short as possible.