Dysplasia in perforated intestinal pneumatosis complicating a previous jejuno-ileal bypass: a cautionary note.

We present the case of an elderly woman who devel- oped a bowel perforation related to pneumatosis intes- tinalis, 33 years after a jejuno-ileal bypass for severe obesity. Final histological examination revealed the presence of dysplasia in the resected specimen. On the basis of our case and a review of the literature, we discuss the etiopathogenesis, the clinical aspects and the treatment of this rare condition

Phase I/II study of single-agent bortezomib for the treatment of patients with myelofibrosis. Clinical and biological effects of proteasome inhibition.

A phase I/II trial was undertaken to determine maximum tolerated dose (MTD), toxicity, clinical efficacy and biological activity of bortezomib in patients with advanced stage primary or post-polycythemia vera/post-essential thrombocythemia myelofibrosis (MF). Bortezomib (0.8, 1.0, or 1.3 mg/m(2)) was administered on days 1, 4, 8, and 11 by intravenous push to patients previously resistant to at least one line of therapy, or with an intermediate/high risk IWG’s score [1]. Therapy was repeated every 28 days for 6 cycles. At 1.3 mg/m(2) dose, one of six patients experienced a dose limiting toxicity, and this was determined to be the MTD. Neither remissions or clinical improvements were recorded in 16 patients treated at this dose level, fulfilling the early stopping rule in the Simon two-stage study design. Major toxicity was on thrombocytopenia. In 9 out of 15 patients bortezomib proved able to reduce bone marrow vessel density. However, the agent was associated with worsening of markers of disease activity, like enhancement of hematopoietic CD34-positive progenitor cell mobilization, WT-1 gene expression in mononuclear cells, and down-regulation of CXCR4 expression on CD34-positive cells. Occurrence of both beneficial and detrimental biological effects claims further investigation on the mechanisms of the drug in MF

Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study

Purpose: To evaluate the clinical features, the prognostic factors, and the efficacy of treatments in patients (pts) with Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) with leukemic presentation. Methods: A retrospective multicenter study was carried out during the period 2005-2011 in 28 Italian hematology divisions among GIMEMA centres. Results: A total of 43 cases were collected (M/F 31/12; median age 68 yo).At diagnosis the median bone-marrow infiltration was 73%; 33 pts (77%) had peculiar skin lesions; lymph nodes and/or spleen involvements were documented in 24 (56%), and extramedullary disease in 9 (21%). In 28 pts (65%) cytogenetic study was performed, revealing an unfavourable karyotype in 12. Forty-one pts received an induction therapy (2 died early), consisting of AML-type regimen in 26 (60%), and ALL/lymphoma-type in 15 (35%); 6 pts (14%) underwent allo-HSCT. Complete remission (CR) was achieved in 17 pts (41%), registering 7 CR after AML-type and 10 after ALL/lymphoma-type regimen, with a significant advantage for ALL/lymphoma-type chemotherapy (p=0.02). The median OS was 8.7 months (range 0.2-32.9):7.1 months (range 0.2-19.5) and 12.3 months (range 1-32.9) in pts received AML-type and ALL/lymphoma-type regimen, respectively (p=0.02). In HSCT-pts the median OS was 22.7 months (range 12-32.9), with a significant advantage with respect to the non-transplanted (median 7.1 months, range 0.2-21.3, p=0.03). Conclusions: BPDCN with bone-marrow involvement is an aggressive subtype of high-risk acute leukemia.With intensive therapy according to ALL/lymphoma-type induction the rate of CR increases. Allo-HSCT performed in first remission may lead to long-term survival in selected cases, but more data are needed

Strategies of the honeybee Apis mellifera during visual search for vertical targets presented at various heights: a role for spatial attention?

When honeybees are presented with a colour discrimination task, they tend to choose swiftly and accurately when objects are presented in the ventral part of their frontal visual field. In contrast, poor performance is observed when objects appear in the dorsal part. Here we investigate if this asymmetry is caused by fixed search patterns or if bees can use alternative search mechanisms such as spatial attention, which allows flexible focusing on different areas of the visual field. We asked individual honeybees to choose an orange rewarded target among blue distractors. Target and distractors were presented in the ventral visual field, the dorsal field or both. Bees presented with targets in the ventral visual field consistently had the highest search efficiency, with rapid decisions, high accuracy and direct flight paths. In contrast, search performance for dorsally located targets was inaccurate and slow at the beginning of the test phase, but bees increased their search performance significantly after a few learning trials: they found the target faster, made fewer errors and flew in a straight line towards the target. However, bees needed thrice as long to improve the search for a dorsally located target when the target's position changed randomly between the ventral and the dorsal visual field. We propose that honeybees form expectations of the location of the target's appearance and adapt their search strategy accordingly. Different possible mechanisms of this behavioural adaptation are discussed.L.M. was recipient of a DOC-fFORTE fellowship of the Austrian Academy of Science at the Department of Integrative Zoology, University of Vienna. L.C. is supported by an ERC Advanced Grant and a Royal Society Wolfson Research Merit Award

[Intrinsic ureteral endometriosis: description of a striking instance.]

Intrinsic ureteral endometriosis is a very rare condition. A 41 y. o. woman with right hydroureteronephrosis and other aspecific symptoms came to our attention. The CT scan showed an ureteral obstacle causing the hydroureteronephrosis. She underwent ureterorenoscopy with biopsies of the lesion that did not result to be diriment. Suspecting a ureteral neoplasm, the patient then underwent ureteral resection and ureterocystoneostomy, and the extemporary histological examination resulted as endometriosis. The abdominal exploration showed a parametrial and a peritoneal growth - both compatible with the extemporary histological examination - that were also excised. The post-operative course was uneventful. The definitive hystological examination confirmed the perioperatory diagnosis. Intrinsic ureteral endometriosis is confirmed as a rare pathology with an indefinite clinical presentation; its typical presentation, namely cyclic hematuria, seems to be an anecdotal feature. Therefore the diagnostics of intrinsic ureteral endometriosis is still difficult even despite such a striking presentation

Standard vs delayed ligature of the dorsal vascular complex during robot-assisted radical prostatectomy: results from a randomized controlled trial

Purpose: Prospective randomized trial to compare standard vs delayed approach to dorsal vascular complex (s-DVC vs d-DVC) in robot-assisted radical prostatectomy (RARP). Methods: Patients scheduled for RARP were randomized into a 1:1 ratio to receive either s-DVC or d-DVC by two experienced surgeons. In s-DVC arm an eight-shaped single stitch was given at the beginning of the procedure and the DVC was subsequently cut at time of apical dissection; in d-DVC arm the plexus was transected at the end of prostatectomy, prior to apex dissection and then sutured. Primary endpoint was difference in estimated blood loss (EBL) and a sample size of 226 cases was calculated; ad interim analysis was planned after 2/3 of recruitment. Results: Endpoint was reached at ad interim analysis after 162 cases (81 s-DVC, 81 d-DVC) and recruitment was, therefore, interrupted. Baseline and tumor characteristics were overlapping. EBL was significantly higher in d-DVC arm (mean EBL 107 vs 65 ml, p = 0.003), but without differences in post-operative hemoglobin, transfusions and complications. Overall PSM rate was higher in d-DVC arm (21.0 vs 14.8%, p = 0.323), with statistical significance relatively to organ-confined disease (15.5 vs 3.6%, p = 0.031). Apical involvement was instead significantly higher in s-DVC arm (prevalence in PSM patients 66.7 vs 23.5%, p = 0.020). Post-operative PSA, continence and potency rates were similar between groups. Conclusions: Standard and delayed approaches to DVC are safe and lead to similar functional outcomes. A delayed approach exposes to a higher risk of PSM in organ-confined disease but with a lower risk of apical involvement