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Lymphomatoid Granulomatosis (LYG) is a rare, EBV-driven disease with angiocentric and angiodestructive pleomorphic lymphocytic infiltrates. Pulmonary involvement is frequent and responsible for pulmonary manifestations which may be associated with systemic symptoms. Its rarity makes it difficult to diagnose, and the diagnosis is usually made after several months of investigation. There is no consensus on treatment, which can range from a waitand- see approach to multidrug therapy with allogeneic hematopoietic stem cell transplantation, according to histological grade. The lack of consensus does not help in the management of these patients. We report the case of a patient with a multirefractory LYG, who achieved a Complete Remission (CR) with gemcitabine as single agent salvage treatment.

The clinical value of imaging is well established for the follow-up of many lymphoid malignancies but not for chronic lymphocytic leukemia (CLL). A meta-analysis was performed with the dataset of 3 German CLL Study Group phase 3 trials (CLL4, CLL5, and CLL8) that included 1372 patients receiving first-line therapy for CLL. Response as well as progression during follow-up was reassessed according to the National Cancer Institute Working Group1996 criteria. A total of 481 events were counted as progressive disease during treatment or follow-up. Of these, 372 progressions (77%) were detected by clinical symptoms or blood counts. Computed tomography (CT) scans or ultrasound were relevant in 44 and 29 cases (9% and 6%), respectively. The decision for relapse treatment was determined by CT scan or ultrasound results in only 2 of 176 patients (1%). CT scan results had an impact on the prognosis of patients in complete remission only after the administration of conventional chemotherapy but not after chemoimmunotherapy. In conclusion, physical examination and blood count remain the methods of choice for staging and clinical follow-up of patients with CLL as recommended by the International Workshop on Chronic Lymphocytic Leukemia 2008 guidelines. These trials are registered at http://www.isrctn.org as ISRCTN 75653261 and ISRCTN 36294212 and at http://www.clinicaltrials.gov as NCT00281918.

Objectives: We sought to assess the smoking policies at state and county fairs in North Carolina. Methods: We contacted North Carolina fair managers by telephone to solicit participation in a survey that assessed the extent to which fairs have written policies about smoking and secondhand smoke (SHS) exposure, managers’ beliefs concerning the health risks associated with SHS exposure, and specific reasons that might prompt managers to adopt smoke-free policies. Results: Attempts were made to contact 47 fair managers, and 31 (66.0%) participated in the study. We found that although almost two-thirds of fairs prohibited smoking indoors, the vast majority (83.9%) had no limits on outdoor smoking. Most fair managers (84.6%) acknowledged that SHS may cause lung cancer, and a majority (51.6%) reported a belief that their patrons would largely be supportive of a more restrictive policy. Limitations: Fair managers’ responses were primarily based on their own opinions, estimates, and attitudes. Conclusions: Because of the high number and density of fair patrons, unrestricted outdoor smoking likely exposes most patrons to SHS. Action to eliminate all exposure to SHS at state and county fairs is needed.

“ The impact of demographic aging within the European Union is likely to become of major significance… The share of those aged 80 years or above is predicted to almost triple between 2011 and 2060 ”. This statement convinced the board of the European Hematology Association (EHA) to define “

Contains fulltext : 137865.pdf (Publisher’s version ) (Open Access) Patients with chronic myeloid leukemia relapsing after allogeneic hematopoietic stem cell transplantation may be treated by tyrosine kinase inhibitors and/or by donor lymphocyte infusions. The best strategies and timing of administration of lymphocytes are unclear. We analyzed 155 patients who relapsed after allogeneic stem cell transplantation with disease detectable only by molecular methods and who subsequently received lymphocytes. Transplants were performed in first chronic phase (n=125) or in advanced disease (n=29) from identical siblings (n=84) or unrelated donors (n=71) between 1986 and 2003. They received lymphocytes either during molecular relapse (n=85) or upon progression to more advanced disease (1993 to 2004). The median interval from relapse to lymphocyte infusion was 210 (0-1673) days. The median follow up after it was 46 (3-135) months. Overall survival was 76+/-4% at five years after lymphocyte infusions (89+/-8% with sibling donors and 63+/-13% with unrelated donors (P=0.003)). Survival was 69+/-14% when lymphocytes were given within six months of the detection of molecular relapse and 81+/-10% (P=0.061) when given later; 81+/-11% if given at molecular relapse versus 71+/-12% (P=0.26) with more advanced disease. In multivariate analysis survival was worse if the donor was unrelated (HR 2.54 (95% CI: 1.15-5.53), P=0.021) and better with lymphocyte infusions beyond six months from molecular relapse (HR 0.4 (95%CI: 0.19-0.84), P=0.018). These data confirm the remarkable efficacy of lymphocyte infusions for this disease. There appears to be no advantage from administering it early upon detection of molecular relapse in patients who received allogeneic stem cell transplantation for chronic myeloid leukemia.

BACKGROUND: Bortezomib and rituximab have shown additive activity in preclinical models of lymphoma, and have been shown to be active and generally well tolerated in a randomised phase 2 study in patients with follicular and marginal zone lymphoma. We compared the efficacy and safety of rituximab alone or combined with bortezomib in patients with relapsed or refractory follicular lymphoma in a phase 3 setting. METHODS: In this multicentre phase 3 trial, rituximab-naive or rituximab-sensitive patients aged 18 years or older with relapsed grade 1 or 2 follicular lymphoma were randomly assigned (1:1) to receive five 35-day cycles consisting of intravenous infusions of rituximab 375 mg/m(2) on days 1, 8, 15, and 22 of cycle 1, and on day 1 of cycles 2-5, either alone or with bortezomib 1·6 mg/m(2), administered by intravenous injection on days 1, 8, 15, and 22 of all cycles. Randomisation was stratified by FLIPI score, previous use of rituximab, time since last therapy, and region. Treatment assignment was based on a computer-generated randomisation schedule prepared by the sponsor. Patients and treating physicians were not masked to treatment allocation. The primary endpoint was progression-free survival analysed by intention to treat. This trial has been completed and is registered with ClinicalTrials.gov, number NCT00312845. FINDINGS: Between April 10, 2006, and Aug 12, 2008, 676 patients were randomised to receive rituximab (n=340) or bortezomib plus rituximab (n=336). After a median follow-up of 33·9 months (IQR 26·4-39·7), median progression-free survival was 11·0 months (95% CI 9·1-12·0) in the rituximab group and 12·8 months (11·5-15·0) in the bortezomib plus rituximab group (hazard ratio 0·82, 95% CI 0·68-0·99; p=0·039). The magnitude of clinical benefit was not as large as the anticipated prespecified improvement of 33% in progression-free survival. Patients in both groups received a median of five treatment cycles (range 1-5); 245 of 339 (72%) and 237 of 334 (71%) patients in the rituximab and bortezomib plus rituximab groups, respectively, completed five cycles. Of patients who did not complete five cycles, most discontinued early because of disease progression (77 [23%] patients in the rituximab group, and 56 [17%] patients in the bortezomib plus rituximab group). Rates of adverse events of grade 3 or higher (70 [21%] of 339 rituximab-treated patients vs 152 [46%] of 334 bortezomib plus rituximab treated patients), and serious adverse events (37 [11%] patients vs 59 [18%] patients) were lower in the rituximab group than in the combination group. The most common adverse events of grade 3 or higher were neutropenia (15 [4%] patients in the rituximab group and 37 [11%] patients in the bortezomib plus rituximab group), infection (15 [4%] patients and 36 [11%] patients, respectively), diarrhoea (no patients and 25 [7%] patients, respectively), herpes zoster (one [

The prospective 1-year Evaluation of Patients' Iron Chelation with Exjade (EPIC) study enrolled a large cohort of 116 patients with aplastic anemia; the present analyses evaluated the efficacy and safety of deferasirox in this patient population. After 1 year, median serum ferritin decreased significantly from 3254 ng/mL at baseline to 1854 ng/mL (P < .001). Decreases occurred in chelation-naive (3229-1520 ng/mL; P < .001, last-observation-carried-forward analysis), and previously chelated (3263-2585 ng/mL; P = .21, last-observation-carried-forward analysis) patients and were reflective of dose adjustments and ongoing iron intake. Baseline labile plasma iron levels were within normal range despite high serum ferritin levels. The most common drug-related adverse events were nausea (22%) and diarrhea (16%). Serum creatinine increases more than 33% above baseline and the upper limit of normal occurred in 29 patients (25%), but there were no progressive increases; concomitant use of cyclosporine had a significant impact on serum creatinine levels. The decrease in mean alanine aminotransferase levels at 1 year correlated significantly with reduction in serum ferritin (r = 0.40, P < .001). Mean absolute neutrophil and platelet counts remained stable during treatment, and there were no drug-related cytopenias. This prospective dataset confirms the efficacy and well characterizes the tolerability profile of deferasirox in a large population of patients with aplastic anemia. This study was registered at www.clinicaltrials.gov as #NCT00171821.

Background Following a clinical evaluation of deferasirox (Exjade) it was concluded that, in addition to baseline body iron burden, ongoing transfusional iron intake should be considered when selecting doses. The 1-year EPIC study, the largest ever investigation conducted for an iron chelator, is the first to evaluate whether fixed starting doses of deferasirox, based on transfusional iron intake, with dose titration guided by serum ferritin trends and safety markers, provides clinically acceptable chelation in patients (agedor=2 years) with transfusional hemosiderosis from various types of anemia.The recommended initial dose was 20 mg/kg/day for patients receiving 2-4 packed red blood cell units/month and 10 or 30 mg/kg/day was recommended for patients receiving less or more frequent transfusions, respectively. Dose adjustments were based on 3-month serum ferritin trends and continuous assessment of safety markers. The primary efficacy end-point was change in serum ferritin after 52 weeks compared with baseline.The 1744 patients enrolled had the following conditions; thalassemia (n=1115), myelodysplastic syndromes (n=341), aplastic anemia (n=116), sickle cell disease (n=80), rare anemias (n=43) and other transfused anemias (n=49). Overall, there was a significant reduction in serum ferritin from baseline (-264 ng/mL; P0.0001), reflecting dosage adjustments and ongoing iron intake. The most common (5%) adverse events were gastrointestinal disturbances (28%) and skin rash (10%). Conclusions Analysis of this large, prospectively collected data set confirms the response to chelation therapy across various anemias, supporting initial deferasirox doses based on transfusional iron intake, with subsequent dose titration guided by trends in serum ferritin and safety markers (clinicaltrials.gov identifier: NCT00171821).

PURPOSE To assess the educational value of an objective structured clinical examination (OSCE) administered in three consecutive years (1992-93 to 1994-95) to first-year residents in a family practice residency. METHOD Each year an OSCE was administered early in a family practice residency based at the University of North Carolina at Chapel Hill School of Medicine. The OSCE encompassed eight to ten stations, each 15 minutes long: ten minutes for tasks and five minutes for feedback. After the OSCE, focus groups with participating faculty and residents assessed the strengths and weakness of specific stations as well as the overall OSCE structure. Each year the residents' OSCE scores were correlated with their scores on the American Board of Family Physicians In-Training Examination (ITE). Pearson product--moment correlation coefficients were calculated for both the composite ITE scores and the clinical set problem scores. Test reliability was measured by Cronbach's alpha. In the spring of 1995 the faculty completed global evaluations of all the residents in the program at the time, including 16 of the 17 residents who had participated in the OSCEs. The faculty rated the residents in four areas, and a four-point scale was based on the percentile ranges a resident's rating could fall into for each area. Correlation coefficients were calculated for the OSCE scores and the faculty's perceptions of the residents' overall performance. RESULTS The OSCE scores correlated significantly with the faculty's global assessments of knowledge and clinical skills, but not with in-training examination scores. Concurrent validity and reliability estimates did not support using an OSCE for decisions about the residents' competency. The faculty valued the opportunity to observe basic skills for the whole group of residents; the residents found the experience educational but stressful and valued the immediate performance feedback included at each station. CONCLUSION The OSCE is a useful tool for teaching basic clinical skills and for forming initial impressions of interns' clinical styles and abilities, but it should be used with caution for summative assessments.

The annual cost of training a single family medicine resident may now exceed $50,000. This, together with the fact that normally only a small fraction of those applying for family medicine residency is selected for admission, creates a decision problem of enormous import to affected institutions. Despite these considerations, the applicant evaluation and selection process remains relatively subjective, with results often disappointing. In the current paper, a preference-based approach is proposed that first models the evaluation/selection process on the basis of well-defined cognitive and noncognitive criteria. It is suggested that validation of this model be based on future performance levels of both the accepted and rejected cohorts during and following their residencies. Discrepancies between future success levels and predicted outcomes may then be translated into appropriate control actions designed to: (1) improve the definition/measurement of selection criteria; (2) enhance the evaluation/selection policies and decisions of the admissions committee; and (3) better inform potential applicants of the department's program and selection philosophies. The approach is applied to two recent, accepted cohorts of the University of North Carolina Department of Family Medicine. Preliminary results indicate that the procedure is capable of improving the in-residency success levels of selected applicants, and that these levels can be better predicted than when no formal, i.e., analytic, process is followed.