[Stable Expression of Coagulation Factors by RPS6 Promoter].

Objective: To screen better promoters and provide more powerful tools for basic research and gene therapy of hemophilia.

Methods: Bioinformatics methods were used to analyze the promoters expressing housekeeping genes with high abundance, so as to select potential candidate promoters. The reporter gene vector was constructed, and the packaging efficiency of the novel promoter was investigated with EF1 α promoter as control, and the transcription and activities of the reporter gene were investigated too.

[Clinical Analysis of Hospitalized Children with Primary Immune Thrombocytopenia].

Objective: To investigate the factors affecting the chronicity of childhood primary immune thrombo-cytopenia (ITP) and compare the efficiency of different first-line treatment regimens.

Methods: Children with ITP hospitalized in our hospital from September 2013 to October 2018 were retrospectively analyzed.

Results: Three hundred and one children (150 males and 151 females) were included in this study, with a median age of 8 (0.

[Symptomatic Profiles of 173 Chinese Patients with Essential Thrombocythemia].

Objective: To explore the symptomatic burden of patients with essential thrombocythemia (ET) and its relation with clinical characteristics including the mutation status, therapeutic protocols and sex.

Methods: Total of 173 Chinese ET patients were selected and grouped on the basis of disease characteristics (mutation status, therapeutic pro to- cols, and sex).

Results: All the groups showed low-to-high symptom burden, with the highest in the Hu (hydroxyurea)-group (total symptom score [TSS], 14.

[Clinical Characteristic of "triple-negative" Essential Thrombocythaemia Patients and Mutation Analysis by Targeted Sequencing].

Background: Essential thrombocythemia is a subgroup of myeloproliferative neoplasms. Previous studies identified mutations of JAK2, CALR, and MPL that are closely related with the pathogenesis of myeloproliferative neoplasms. All these mutations contribute to the hyperactivation of JAK2/STAT pathway.

Chinese guidelines for treatment of adult primary immune thrombocytopenia.

Primary immune thrombocytopenia (ITP) is a bleeding disorder commonly encountered in clinical practice. The International Working Group (IWG) on ITP has published several landmark papers on terminology, definitions, outcome criteria, bleeding assessment, diagnosis, and management of ITP. The Chinese consensus reports for diagnosis and management of adult ITP have been updated to the 4th edition.

[Clinical Characteristics and Long-Term Outcome of 125 Chinese Young Patients with Essential Thrombocythemia].

Objective: To investigate the clinical characteristics and long-term outcome of Chinese young patients (≤40 years) with essential thrombocythemia(ET), and to develop a thrombosis predicting model specific for young patients with ET, so as to provide a new evidence for risk stratification and treatment.

Methods: Medical records of 125 Chinese young patients with newly diagnosed of ET were retrospectively analyzed.

Results: The median age at diagnosis was 32 (18-40) years old, with 37 males and 88 females.

[Role of Toll-like receptor 2 in primary immune thrombocytopenia].

The aim of this study was to explore the role of Toll-like receptor (TLR) 2 in primary immune thrombocytopenia (ITP) by detecting TLR2 expression in the peripheral blood lymphocytes of patients with ITP and evaluating the role of TLR2 activation on inflammatory cytokine secretion. A total of 39 ITP patients and 21 normal controls were enrolled in this study. The expression of TLR2 was detected by real-time PCR and flow cytometry, and the concentration of IL-6 and TNF-α in culture supernatant of PBMNC treated with pam3CSK4 for 48 hours were detected by ELISA.

Interleukin-7 is decreased and maybe plays a pro-inflammatory function in primary immune thrombocytopenia.

Primary immune thrombocytopenia (ITP) is an autoimmune disease with many immune dysfunctions, including over-proliferation and apoptosis resistance of auto-reactive lymphocytes. This study aimed to determine the effects of interleukin (IL)-7 on the cytokine production and survival of peripheral blood mononuclear cells and bone marrow mononuclear cells from ITP patients. We found that the plasma IL-7 levels in peripheral blood from ITP patients were lower than that of the normal controls, and it had positive correlation with platelet counts.

[Clinical characteristics of 520 children with hemophilia in a single medical center].

Objective: To analyze the clinical characteristics, diagnosis and treatment of pediatric hemophilia in single center over the decade.

Methods: A retrospective study was conducted with 520 hemophilic children hospitalized in our medical center between January 2002 and December 2012.

Results: All the patients were male including 438 hemophilia A (HA) and 82 hemophilia B (HB).

[Relationship between the expression of autoantibodies against platelet membrane glycoprotein and therapeutic effect in primary immune thrombocytopenia].

Objective: To study the expression of specific anti- platelet glycoprotein autoantibodies GP II b/III a, GP I b/IX and GP I a/II a in primary immune thrombocytopenia (ITP), and to evaluate the relationship between the therapeutic effect and the expression of specific anti- platelet glycoprotein antibodies GPIIb/IIIa, GPIb/IX and GPIa/IIa.

Methods: Anti-GPIIb/IIIa, GPIb/ IX and GP I a/II a antibodies were assayed by ELISA for patients with ITP. Total 442 patients in our hospital, who were retrospectively investigated from December 2010 to November 2012, were divided into newly diagnosed ITP, persistent and chronic ITP.

[Acquired factor V inhibitor: 3 cases report and literature review].

Objective: To better understand the acquired factor V (FV) inhibitors.

Methods: The clinical features, laboratory manifestations, treatment options and prognosis of 3 cases were reported and related literature were reviewed.

Results: All the 3 patients were older than 50 years without family history and related disease.

[A novel diagnostic measure of platelet-specific antibody in immune thrombocytopenia].

Objective: To detect the platelet glycoprotein-specific antibodies in serum of thrombocytopenia patients and evaluate its diagnostic value for immune thrombocytopenia.

Method: Anti-GPIIb/IIIa, GPIb/IX and GPIa/IIa antibodies were assayed by ELISA kit (PAKUTO) in patients with thrombocytopenia.

Results: The sensitivity and specificity of PAKAUTO in immune thrombocytopenia were 44.

[Effect of mesenchymal stem cells on human Th1 cells by flow cytometry].

This study was aimed to investigate the effect of fetal bone marrow-derived mesenchymal stem cells (FBM-MSC) on the development of human Th1 cells. FBM-MSC were isolated, cultured and expanded in vitro. The cells were identified by their phenotype profiles and differential capacity.

[The activity levels and prevalence of deficiency of protein C, protein S and antithrombin in Chinese Han population].

Objective: To explore the distribution and influence factors of protein C (PC), protein S (PS) and antithrombin (AT) activities and to determine the prevalence of their deficiencies in the Chinese Han healthy population.

Methods: Healthy volunteers including blood donors and individuals for routine check-up were recruited from 4 Chinese medical centers. The plasma levels of PC, PS and AT activities were measured.

[The role of Th17 cells in immune thrombocytopenia mice models].

Objective: To investigate the role of Th17 cells in immune thrombocytopenia (ITP) mice model.

Method: ITP was induced by daily intraperitoneal injection of anti-platelet membrane CD41 antibody (MWReg30) into BALB/c mice, the mRNA expressions of Th17 cell associated transcription factors and cytokines in peripheral blood and spleen mononuclear cells were measured by real-time PCR, and the proportion of Th17 cells by FCM analysis.

Results: The percentage of Th17 cell was significantly elevated in ITP mice both in splenocyte and peripheral blood as compared with that in normal controls (P<0.

[Efficacy and safety of two different low-dose rituximab regimens for Chinese adult patients with immune thrombocytopenia].

Objective: To compare the efficacy of two different regimens of low doses rituximab for the treatment of adult patients with immune thrombocytopenia (ITP).

Methods: Fifty-one patients were enrolled in this study and was non-randomly assigned to receive 100 mg rituximab weekly for 4 weeks (group A, 31 cases) or a single dose of 375 mg/m2 rituximab (group B, 20 cases).

Results: For group A: Overall and complete response (OR and CR) rates were 58% and 29% , respectively.

Elevated interleukin-27 enhances the polarization of Th1/Tc1 cells and the production of proinflammatory cytokines in primary immune thrombocytopenia.

Primary immune thrombocytopenia (ITP) is an acquired, organ-specific, autoimmune disease with many immune dysfunctions. Interleukin-27 (IL-27) can regulate T cell differentiation. However, it is unclear whether IL-27 correlates with the dysfunctions of T cell differentiation in ITP patients.

CD72 gene expression in immune thrombocytopenia.

To explore the role of CD72 in the pathogenesis of immune thrombocytopenia (ITP), we detected CD72, Sema4D, IL-2, IL-4, and IFN-γ mRNA expressions and the levels of plasma Sema4D, IL-2, IL-4, IL-6, and IFN-γ in ITP patients (n = 39) and controls (n = 23). The levels of plasma IL-2, IL-4, and IL-6 were assayed by radioimmunoassay, and the levels of plasma IFN-γ and Sema4D were analyzed by enzyme-linked immunosorbent assay. Sema4D, CD72, IL-2, IFN-γ, and IL-4mRNA expressions were analyzed by real-time quantitative reverse-transcription polymerase chain reaction.