Lundgrenthurston2055

ear OS rate of grade Ⅲ aGVHD group was superior to grade Ⅳ aGVHD group [ (84.2±8.4) % vs. (47.6±13.1) % , χ(2)=3.38, P=0.05]. Conclusion This study demonstrated that P-ATG with etanercept was effective and safe in treating grade Ⅲ-Ⅳ aGVHD after allo-HSCT.Objective This study aims to investigate the expression of E3 ubiquitin-ligase (WWP1) in chronic lymphocytic leukemia (CLL) patients and analyze its correlation with clinical prognostic indicators (TP53, CD38, IGHV mutation) and its prognostic value. Methods A total of 48 CLL patients and 9 age-matched normal subjects were enrolled in the study. The WWP1 expression was detected by SYBR Green-based real-time PCR, and the clinical relationship was analyzed by GraphPad Prism software. buy CPI-613 Results The WWP1 median expression was 0.007 (95% CI 0.005-0.010) in the normal control group and 0.031 (95% CI 0.019-0.044) in the CLL group (P less then 0.001) . A sub-groups analysis implicated a statistically significant result (P=0.022) , showing that the median time from a relatively high and low transcription level of WWP1 to the first treatment was 24 months and 35 months, respectively. Positive CD38 and ZAP-70 expressions were associated with a higher WWP1 expression (P=0.012 and 0.029, respectively) . Conclusion An abnormal WWP1 mRNA expression was found in CLL patients with significant correlation with ZAP-70 and CD38 expressions, and WWP1 may become a new supplement of CLL prognostic markers.Objective This study aims to investigate the characteristics of gene mutation and clinical prognosis in de novo acute myeloid leukemia (AML) patients with myelofibrosis (MF) . Methods From January 1, 2016, to February 1, 2020, 103 newly diagnosed AML patients in Henan Provincial People's Hospital who simultaneously underwent bone marrow biopsy examination were included. They were divided into the AML-MF group (MF grades 1-3) and the AML without MF group (MF grade 0) , and the clinical features, gene alterations, chemotherapy efficacy, and prognosis were compared between the two groups retrospectively. Results ①MF was confirmed in 44.7% of AML patients (46/103) , of which 84.8% (39/46) were MF-1 and 15.2% (7/46) were MF-2/3, while MF was not confirmed in 55.3% (57/103) of AML patients. The median of WBC in the AML-MF group was significantly higher than in the AML without MF group [11.205 (0.69-191.82) ×10(9)/L vs 4.64 (0.18-95.10) ×10(9)/L, P=0.024]. More patients in the AML-MF group had nucleated erythrocytes0.044) . Conclusion AML-MF has unique laboratory and clinical characteristics. MF is an independent risk factor for CR, OS, and PFS in AML. Evaluation of MF is very significant for therapy efficacy and prognosis judgment in de novo AML.Objective To explore the molecular features and prognostic value of RAS mutations in patients with myelodysplastic syndromes (MDS) . Methods 112-gene targeted sequencing was conducted to detect RAS mutations in 776 patients with newly diagnosed primary MDS from December 2011 to December 2018. The mutual exclusivity and co-occurrence in gene mutations and clonal architecture were explored. Moreover, the prognostic significance of RAS mutations in MDS was analyzed. Results RAS gene mutations were found in 52 (6.7% ) cases, 38 (4.9% ) of whom harbored NRAS mutation, 18 (2.3% ) KRAS mutation, and 4 (0.5% ) both NRAS and KRAS mutations. All the NRAS mutations and 65% of the KRAS mutations were located in codons 12, 13, and 61. PTPN11, FLT3, U2AF1, RUNX1, WT1, ETV6, and NPM1 mutations were enriched in patients with RAS mutations (Q less then 0.05) . Around 80% of RAS mutations represented subclonal lesions in patients who harbored at least two different mutations. Patients with RAS mutations were more frequently diagnosed with MDS with excess blast (MDS-EB) (82.7% vs. 35.2% , P less then 0.001) and had higher levels of white blood cell count (4.33×10(9)/L vs. 2.71×10(9)/L, P less then 0.001) , neutrophil absolute count (2.13×10(9)/L vs. 1.12×10(9)/L, P less then 0.001) , and bone marrow blast percentage (7% vs. 2% , P less then 0.001) but lower levels of platelet count (48×10(9)/L vs. 62×10(9)/L, P=0.048) . RAS mutations were correlated with higher-risk categories in the Revised International Prognostic Scoring System (IPSS-R) (71.1% vs. 37.9% , P less then 0.001) . The median overall survival of patients with NRAS mutations was shorter than the others (P=0.011) , while the significance was lost in the multivariable model. Conclusion RAS gene mutations always occurred in the late-stage MDS and co-occurred with other signal transduction- and transcription factor-related gene mutations. PTPN11, a RAS pathway-related gene, is an independent poor prognostic factor in MDS patients.Objective To observe the efficacy and safety of sirolimus combined with calcineurin inhibitor (CNI) in the treatment of glucocorticoid resistant/dependent extensive chronic graft-versus-host disease (cGVHD) . Methods A total of 27 patients with steroid-resistant/steroid-dependent extensive cGVHD from November 2015 to January 2019 were enrolled and given sirolimus capsules combined with cyclosporine or tacrolimus to observe the clinical efficacy and adverse events. Results The median duration of medication was 14.2 months and the mean duration was 16.7 months. The median follow-up time was 20.1 months (12.9-46.1 months) . Following the 6-month follow-up, 3 cases achieved complete response (CR) and 12 cases partial response (PR) . The overall response rate (ORR) was 55.6% ; for progression-free survival (PFS) , PFS-6 reached 88.9% (24/27) , and for overall survival (OS) , OS-6 was 100% . At the 1-year follow-up, there were 5 cases of CR and 11 cases of PR, ORR was 59.3% , PFS-12 reached 62.9% (17/27) , and OS-12 was 100% . The subgroup analysis found that the program was more effective for cGVHD in male donors and the target organ analysis had an advantage in the treatment of oral cavity, skin, and liver rejection. Adverse events were observed hyperlipidemia 11.1% , oral ulcer 7.4% , fungal infection 11.1% , liver injury 3.7% , renal insufficiency 0, and no new CMV and EB viremia. Conclusion Sirolimus combined with calcineurin inhibitors is effective in treating steroid-resistant/steroid-dependent extensive cGVHD, especially because adverse reactions (renal toxicity, CMV, EBV infection) are low in number, which is suitable for long-term treatment of cGVHD.