Background & Aims. At present, the allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the only treatment option with curative potential in patients with myelofibrosis (MF), especially in intermediate and high risk categories. The aim of the study is to perform a retrospective analysis of alloHSCT outcomes in MF patients. Materials & Methods. Outcomes of allo-HSCT in 11 intermediate-2 (n = 3) and high (n = 6) risk patients (based on Dynamic International Prognostic Scoring Scale, DIPSSplus) performed in the R.M. Gorbacheva Scientific Research Institute of Pediatric Hematology and Transplantation over the period from 2005 till 2015 were analyzed in the study. Two more patients underwent allo-HSCT in MF blast phase. Two patients received ruxolitinib before allo-HSCT and 1 patient before and after allo-HSCT. Reduced intensity conditioning regimen was used in all cases. Results. Primary engraftment was documented in 8 patients. 72 % of patients achieved complete hematological remission. Molecular remission and myelofibrosis regression were confirmed in 5 patients. 5 of 11 patients were still with remission and followed-up by the date of the paper submission. The overall two-year survival was 46 %. Conclusion. Allo-HSCT is an effective treatment option for MF patients. Further trials are required to evaluate an optimal timing for allo-HSCT in MF patients and efficacy of Janus kinase (JAK) inhibitors as pre- and posttransplant therapy in MF.
Myocardial damage in patients with chronic renal failure histologically manifests as hypertrophy of cardiomyocytes, intramyocardial artery walls, development of diffuse sclerosis and a change in the number of capillaries. One of the components in the complex treatment of this category of patients is a low-protein diet, however, to date there is no data on the effect of a low-protein diet on structural changes in the myocardium. The aim of the work was to assess the effect of low protein diet on myocardial structural changes in the experimental model of chronic renal failure. To reproduce the experiment of chronic renal failure, a standardized 5/6 nephrectomy model was used in wistar rats. To determine the effect of a low-protein diet on structural changes in the myocardium, two types of diet were used - a standard one and a low-protein diet (Ketosteril). The animals were sacrificed 4 months after nephrectomy. For a comparative assessment of the effect of low-protein diet on structural changes in the myocardium, histological methods of investigation and morphometry were used. The use of low-protein diet was accompanied by a less pronounced violation of biochemical blood parameters (creatinine, urea, calcium, phosphorus), as well as maintaining normal blood pressure, myocardial mass and left ventricular wall thickness. Histologically, this was manifested by a decrease in the severity of dystrophic changes in cardiomyocytes, the degree of their hypertrophy, a decrease in the area of nuclei, and a decrease in the nuclear-cytoplasmic ratio. Indices of perivascular and diffuse sclerosis were lower compared with the control group. Also, when using low-protein diet in animals with a chronic renal failure model, a decrease in the total cross-sectional area of capillaries was noted with an increase in their number in comparison with animals of the control group. Thus, the use of a low-protein diet in an experimental model of chronic renal failure has a cardioprotective effect by reducing the severity of uremia and stabilizing biochemical parameters.
patients with PTCL were treated and included in the analysis. Overall survival (OS) was calculated from the time of diagnosis to death from any cause. Kaplan-Meier curves were generated and compared based on the log-rank test. Cox proportional hazard models were used to investigate the association by adjusting for age, gender, diagnosis, and the type of treatments. The analysis was done in SAS version 9.4. Table 1 summarizes the subtypes of PTCL, median age, and median OS. First line therapies mostly consisted of CT such as CHOP, CHOEP, and EPOCH. Patients who did not achieve a complete remission (CR) with first line CT were at increased risk of death (HR 4.6, p<0.0001). This remained true after excluding anaplastic large cell lymphoma (ALCL), which has a better prognosis (HR 4.0, p=0.0001). Second and third line therapies included platinum-and gemcitabine-based CT, clinical trials, and novel agents. Results:Importantly, patients who did not achieve a CR with second line novel agents had increased risk of death compared to those who achieved CR (HR 11, p=0.019) as shown in Figure 1. Patients who did not undergo autologous or allogeneic stem cell transplant (ASCT) had increased risk of death (HR 1.2, p=0.0058), and this impact was stronger after removing patients with ALCL from analysis (HR 2.5, p=0.016). Conclusions:Our data is consistent with historical data, where ALK+ ALCL did better compared to other subtypes of PTCL.Patients who received NA as second line therapy after relapse and achieved CR had a survival benefit. Novel agents can be efficacious in a the relapsed/refractory population with limited treatment options. We are currently finding new ways to combine NA upfront to shift the paradigm in the treatment of these heterogeneous diseases.ABSTRACT 473
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