With advances in medical sciences,immune thrombocytopenia (ITP) is gradually regarded as one of diseases characterized bv underlying loss of immune tolerance.Accordingly,treatment methods and strategy are shifting from traditional clinical experience to targeted therapy on a step-by-step basis.As for chronic and refractory ITP,combination therapy,novel immunological targeting drugs are turning out to be more effective treatment options.With further understanding of ITP pathogenesis,the elucidation of infections in the development of childhood ITP,the realization that ITP is in fact an immunological syndrome,and the distinction of other diseases with ITP-like clinical manifestations,are of great clinical implications.

OBJECTIVE:To explore appropriate pharmaceutical care methods for hemophilia children,and to improve rational drug use. METHODS:Drug consultation hemophilia records collected from our hospital during Nov. 2014 to Jul. 2015 were summa-rized in respects of personal structure,form and content;key cases were analyzed specially. RESULTS:During Nov. 2014-Jul. 2015,clinical pharmacists received 130 times/cases of medication consultation that related to hemophilia in our hospital;patient’s family members occupied the main part (70.0%);most of drug consultation form was information platform for consultation (58.5%);consultation content mainly covered drug combination and auxiliary medication (77.7%). Clinical pharmacists have re-duced the risk of bleeding,improved the quality of life,reduced the economic burden of families and strengthened the awareness of medical staff and patient’s family member on safe drug use through finding out medication error timely,paying attention to the component of compound drug,guiding medication direction and optimizing therapy plan. CONCLUSIONS:Clinical pharmacists ac-tively provide pharmaceutical care and promote rational drug use during hemophilia treatment from medication consultation,ratio-nal drug use,etc.

Objective:To explore the inhibitory effect of arsenic troixide (ATO) on the growth of human endometrial cancer HEC-1-A cells in vitro and in vivo. Methods:Tetrazolium salt assay (MTT) was used to compare the inhibitory effect of ATO on HEC-1-A cells with that of progesterone, medroxyprogesterone acetate (MPA) and cisplatin (CDDP). Flow cytometry and DNA electrophoresis were used to determine the effects of ATO on cell cycle and apoptosis. Human endometrial cancer xenografted model was established in nude mice. The tumor-bearing nude mice were randomly divided into the experimental groups: ATO low dose group (4 mg·kg-1·d-1), medium dose group (6 mg·kg-1·d-1), high dose group (8 mg·kg-1·d-1), CDDP positive control group (3 mg·kg-1·d-1) and saline negative control group. The drugs were administered intraperitoneally for 14 consecutive days, and then the tumor volume and tumor inhibition rate were calculated. Results: ATO 1-20 μmol/L and CDDP markedly inhibited the cell growth. The inhibitory effect of ATO was higher than that of CDDP. ATO 5 μmol/L treatment induced apoptosis and arrested cells at S and G2/M phase. ATO 4, 6, and 8 mg·kg-1·d-1 and CDDP 3 mg·kg-1·d-1 inhibited tumor volume by 50.97%, 75.58%, 56.92%, and 52.23%, respectively; and inhibited the tumor weight by 10.15%, 29.33%, 16.67%, and 14.69%, respectively. The difference was significant compared with negative control group (P<0.05). Conclusion:ATO inhibited the growth of endometrial cancer cells HEC-1-A in vitro and in vivo. It may become a novel therapeutic reagent for the treatment of endometrial cancer.

Objective To investigate the impact of auto and allogenic mesenchymal stem cells (MSC) transplantation on hematopoietic reconstitution. Methods MSC from auto, donor bone marrow or embryonic tissue were cultured and expanded in vitro in the serum culture system. Five patients received hematopoietic stem cell transplantation (HSCT) were investigated. Case 1 of systemic lupus erythematosus and Case 2 of non-hodgkin' s lymphoma (NHL) received auto MSC transplant before auto-HSCT. Case 3 of paroxysmal nocturnal hemoglobinuria received HLA-identical allogenic MSC transplant before HLA-identical allo-HSCT.Case 4 of chronic myelocytic leukemia and Case 5 of NHL had delayed hematopoietic reconstitution (129th and 78th day, respectively) after allo- and auto-HSCT, respectively, and received MSC from embryonic tissue.Results Case 1, 2 and 3 had no manifested side effects after MSC transplantation combined with HSCT.Neutrophil count of case 1, 2, and 3 were over 0.5 ×109/L at 1st, 10th and 10th day, respectively, platelet count were over 20 ×109/L at 1st, 8th and 33th day, respectively, and agranulocytosis at Ost, 7th and 12th day, respectively. The treatment of embryonic tissue MSC transplant was confirmed to fail for Case 4 and 5.Conclusion The time of MSC transplant has a great impact on hematopoietic reconstitution. MSC transplantation and HSCT performed simultaneously can improve hematopoietic reconstitution. However, the impact of MSC on patients with delayed hematopoietic reconstitution after HSCT needs further study.

Objective To analyze the clinical characteristics of juvenile myelomonocytic leukemia(JMML) and the PCNA levels of the hyperoxia -exposure group (6 h)decreased,and the difference in PCNA protein expres-sion levels was significant of gene diagnose for JMML.Methods Clinical data were retrospectively analyzed in 21 pa-tients suffering from JMML based on new 2009 World Health Organization diagnostic criteria from January 201 3 to June 201 4 in Beijing Children′s Hospital,Capital Medical University.Results There were 85.7% (1 8 /21 cases)patients within 4 -year -old children,and the median age was 23 months (2 -86 months).Fever and abdominal symptoms were the prominent clinical symptoms,52.4% (1 1 /21 cases)with fever,38.1 % (8 /21 cases)with abdominal dis-tention,diarrhea and other abdominal symptoms,80.5% (1 9 /21 cases)had splenomegaly (mild 1 9.1 %,middle 33.3%,severe 38.1 %),and some patients had other tissue infiltration,such as rash,yellow tumor and lymphnode enlargement.Peripheral blood cell count showed that the white blood cells increased because of anemia or thrombocyto-penia,ranging from 1 0.40 ×1 09 /L to 82.1 4 ×1 09 /L(median,26.1 0 ×1 09 /L),and the monocyte counts ranged from 1 .46 ×1 09 /L to 21 .60 ×1 09 /L(median,3.79 ×1 09 /L),characteristics of JMML gene abnormality was detected in 1 7 cases:including 1 1 single gene mutation,and 6 cases with double gene mutations.PTPN11 was the highest frequency of occurrence,accounting for 52.9% (9 /1 7 cases),and NF1 mutation was 35.3% (6 /1 7 cases).All the patients were followed up by phone call,the median follow -up time was 371 days (57 -562 days),6 patients were lost to follow -up,7 patients died,2 patients were alive after hematopoietic stem cell transplantation,1 patient converted to acute non lymphocytic leukemia,and 5 patients were still alive after receiving symptomatic treatment.Among dead cases,PTNT11 gene mutation and NF1 gene mutation were detected in 6 patients;among living children after hematopoietic stem cell transplantation,RAS and PTNT11 mutations were detected in 2 patients;among living children after symptomatic treat-ment,RAS mutation was detected in 2 patients and PTPN11 +CBL mutation in 1 case.Conclusions The symptom and laboratory examination of JMML have no specificistics,with poor prognosis,gene diagnose has guiding significance for JMML diagnose and for selecting therapy.

Objective To investigate the expression of syntaxin 8(STX8)in glioma and its clinical signif-icance. Methods Specimens of glioma were collected from 57 patients at Beijing Renhe Hospital from May 2013 to December 2015. 57 pieces of glioma tissue were used as a study group ,12 of which were Ⅰ+ Ⅱ(low grade) and the rest 45 were Ⅲ+Ⅳ;normal brain tissues from 15 individuals were used as a control group. Real-time PCR,immunohistochemistry,and Western blot were used to detect expression of STX8. Results As compared with the normal brain tissue ,the mRNA expression of STX8 was significantly increased in glioma tissue ,with a relative expression volume of 1.6855 ± 0.07124 in low grade and 2.8207 ± 0.0692 in high grade tissues,there was significant differences between the two groups;and the difference was also significant as compared with the control group(P < 0.05). The results of immunohistochemistry showed that the expression of STX8 was higher in glioma tissue than in normal tissue. Western Blot showed that the expression of STX8 protein was significantly higher in glioma than in normal tissue(P<0.05);the relative expression volume of STX8 was 2.271 ± 0.1621 in low grade tissue and 4.937 ± 0.1851 in high grade tissue,with a significant difference between the two groups;the difference was also significant as compared with the control group(P<0.05). The correlation analysis showed that higher STX8 expression in glioma was not significantly related to gender,age and pathological types,but there was a significant difference between pathological stages. Conclusion STX8 has abnormal high expression in glioma,which may be closely related with the occurrence and development of glioma.

The clinical and laboratory data of a patient with chronic refractory immune thrombocytopenia (ITP) who had a significant increase in the proportion of inhibitory T cells in the hematological oncology center of Beijing Children′s Hospital Affiliated to Capital Medical University in February 2018 and regularly followed up in the outpatient department received a retrospective analysis.This 8-year-old patient′s clinical features were 6 years of skin and mucous membrane bleeding spots and petechiae recurring, with occasionally nasal epistaxis.Physical examination: cardiopulmonary abdomen and nervous system examinations are normal, and no superficial lymphadenopathy is touched.Blood routine indicated that the platelets were 2.00×10 9/L, and white blood cell count and hemoglobin level were normal.Bone marrow suggested that hyperplasia was significantly active, with more than 300 megakaryocytes.The patient was diagnosed with ITP, and he was treated with first-line treatment with gamma globulin and oral Corticosteroids.The first-line treatment with high-dose Dexamethasone therapy was repeated, and the second-line treatment was low-dose Rituximab combined with high-dose Dexamethasone.Evaluations had been conducted with every relapsed course, and Cyclosporine was administered orally on the basis of the highly suppressive T cells.After half a month, the child achieved partial remission and continued for 3 months, and then reached a complete remission of 6 months (till this paper). It is suggested that, with ITP as an immunological disease with high heterogeneity, the immune abnormality index is expected to become a breakthrough in the development of precise treatment.

Objective To investigate the distribution of TCR Vβ genealogy and clonal expansion in peripheral blood after infusing mesenchymal stem cells (MSC) in patients with chronic GVHD. Methods The complementarity determining region 3 (CDR3) of 24 TCR Vβ subfamily genes in peripheral blood mononuclear cell from 1 case with cGVHD after allogeneic hematopoietic stem cell transplantation (Allo-HSCT),who were treated with infusing MSC,were amplified using RT-PCR. The blood samples were taken at the first and the fifth day after 1st infusion; and the first day,the 10 th day and the 20 th day after the second infusion of MSC,as well as the MSC infused as control . The products were labelled by fluorescein and then analyzed the CDR3 size with gene scan technique to determine the clonality of T cells. Results There were no expression of TCR Vβ subfamily with the MSC infused and after the 1st day of the first infusion of MSC. Then 3,10,14,10 Vβ subfamilies clones are appeared at the other time points,of which were polyclone and oligoclone predominately. In the same time,the manifestations of cCVHD have been abated. Conclusion MSC played a certain role in reviving the immune function of the patients after Allo-HSCT and mitigating the disease of chronic GVHD. Lineage analysis of TCR Vβ subfamily showed some predominant expression.

Objective To analyze the diagnostic status of haemophilia in Chinese children in recent years,and to provide information for increasing the life quality of children with haemophilia in China.Methods The pediatric haemophilia cases registration data were collected and analyzed by using questionnaire,from January 1,2008 to March 30,2014 in 13 haemophilia treatment centers of haemophilia treatment center collaboration network of China pediatric group.These centers were from 12 provinces / municipalities.Results A total of 554 cases were collected;median age was 7.0 years old(0.1 to 17.9 years old);among them,481 cases(86.8％) were hemophilia A,and 73 cases were hemophilia B (13.2％);55 mild cases (9.9％),290 moderate cases (52.4％),and 209 severe cases (37.7％);162 cases(29.2％) had family history,the other 392 cases(70.8％) had no family history.The diagnosis was made at a median age of 12.0 month-old(0 to 180.0 months);the diagnosis time was 0.5 months(0 to 144.0 months) after the first bleeding;diagnosis timing with short interval was in 356 cases(64.3％),long interval was in 198 cases(35.7％).The diagnostic timing was not correlated with disease severity (P =0.812) or the family history (P =0.243);but correlated with the severity of first bleeding(P =0.027) and per capita gross domestic product (P ＜ 0.01) in patients' residence.From 1996 to 2013,the annual number of newly diagnosed cases was increasing year by year,with a higher proportion of short interval of diagnose timing.Conclusions With development of hemophilia work in China,the number of diagnosis of haemophilia children is increasing year by year.Moderate and severe hemorrhage are both taken seriously and diagnosed timely.But the diagnosis is delayed in some children.Chinese haemophilia work still need to be strengthen and the propaganda and diagnostic technology are to be popularized.