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Median leukemia-free survival was 8.7 months, and median overall survival was 15.2 months. Median leukemia-free survival and overall survival were not reached in patients proceeding to alloSCT compared to 5.1 and 15.2 months, respectively, for patients who did not receive stem cell transplantation.Treatment was well tolerated with neurological events reported in two patients (10%) and GI events in three patients (14%). Cytokine storm was reported in four patients (19%).In conclusion, treatment with blinatumomab is effective and tolerable in adult patients with relapsed/refractory B-ALL outside of a clinical trial stetting.Pulmonary hypertension (PH) has been described in myelofibrosis (MF), but it is rare and typically found in advanced disease. Although the etiology of PH in MF is unclear, early predictors may be detected by echocardiogram. The goals of our study were to evaluate the prevalence of PH as determined by echocardiography in a cohort of MF patients and to identify clinical risk factors for PH. We performed a retrospective review of MF patients from October 2015 to May 2017 at MD Anderson Cancer Center in the ambulatory clinic, and those with echocardiogram were included. Clinical, echocardiographic, and laboratory data were reviewed. GW 501516 Patients with and without PH were compared using a chi-square or Fisher's exact test, and logistic regression was performed with an outcome variable of PH. There were 143 patients with MF who underwent echocardiogram, and 20 (14%) had echocardiographic findings consistent with PH. Older age, male gender, hypertension, hyperlipidemia, coronary artery disease, dyspnea, hematocrit, brain natriuretic peptide (BNP), and N-terminal prohormone BNP (NT-proBNP) were significantly different between those without PH and those with PH (p less then 0.05). Female gender was protective (OR 0.21, 95% CI 0.049-0.90, p = 0.035), and NT-proBNP was a significant clinical predictor of PH (OR 1.07, CI 1.02 = 1.12, p = 0.006). PH in MF is lower than previously reported in our MF cohort, but many patients had cardiac comorbidities. PH due to left-sided heart disease may be underestimated in MF. Evaluation of respiratory symptoms and elevated NT-proBNP should prompt a baseline echocardiogram. Early detection of PH with a multidisciplinary approach may allow treatment of reversible etiologies.The article "In the South African setting, HIV-associated Burkitt lymphoma is associated with frequent leukaemic presentation, complex cytogenetic karyotypes, and adverse clinical outcomes".PURPOSE Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome emerging from a deregulated immune response due to various triggers. In adults, systematic data are sparse, which is why recommendations on diagnosis and management have been adopted from pediatric guidelines. A nationwide clinical registry with associated consulting service as collaborative initiative of HLH-specialized pediatricians and hematologists was initiated to better characterize HLH in adults. METHODS Patients with proven or suspected HLH were registered by 44 institutions. Both HLH-2004 diagnostic criteria and the HScore (www.saintantoine.aphp.fr/score/) were used to confirm HLH diagnosis. Data referring to underlying disease, treatment, outcome, clinical presentation and laboratory findings were recorded. RESULTS The study included 137 patients and provides the first systematic data on adult HLH in Germany. Median age was 50 years with a wide range (17-87 years), 87 patients (63.5%) were male. Most common triggering diseases were infections in 61 patients (44.5%) and malignancies in 48 patients (35%). Virtually all patients had elevated ferritin concentrations, and 74% had peak concentrations greater than 10,000 µg/l. At time of analysis, 67 of 131 patients (51%) had died. Patients with malignancy-associated HLH had the shortest median survival (160 days), however no statistically significant difference between subgroups was observed (p = 0.077). Platelets under 20*109/l and low albumin concentrations ( less then 20 g/l) were associated with poor overall and 30-day survival. CONCLUSION Close multidisciplinary case consultation and cooperation is mandatory when treating adult HLH patients. Early contact with reference centers is recommended, especially in relapsing or refractory disease.PURPOSE To identify independent risk factors for urethroplasty failure in a prospective dataset. METHODS Since 2008, data of all male patients undergoing urethroplasty at Ghent University Hospital have been prospectively recorded and maintained. This analysis excluded posterior strictures, strictures of the perineostomy, urethral malignancy-related strictures, age less then 18 years and follow-up less then 1 year. Postoperatively, a voiding cysto-urethrography (VCUG) was performed after 2 weeks and in absence of significant contrast extravasation, the transurethral catheter was removed. Patients were followed after 3 m, 12 m and annually thereafter. Failure was defined as stricture recurrence requiring additional urethral intervention(s). Uni- and multivariate Cox regression analyses were performed on the entire patient cohort and for one-stage urethroplasty (OSU) at specific locations. RESULTS In total, 474 patients were included. Median follow-up was 62 m (IQR 35-91). Significant extravasation was present in 6.9%. Bulbar stricture location was identified as independent protective factor for urethroplasty failure (HR 0.44; p = 0.046) and significant extravasation at first VCUG was identified as independent risk factor for urethroplasty failure (HR 2.86; p = 0.005). Cox regression analyses for OSU at specific locations could not identify other risk factors. All but one (89%) of the failures preceded by significant extravasation at first VCUG occurred within 2 years of follow-up whereas 44% of the failures with no or insignificant extravasation at first VCUG occurred after 2 years of follow-up (p = 0.03). CONCLUSIONS Bulbar stricture location is an independent protective factor for urethroplasty failure. Significant extravasation at first urethrography is an independent risk factor for urethroplasty failure and is associated with earlier stricture recurrence than other failed cases.
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