Evans syndrome (ES) is a rare disease characterized by the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) and/or immune neutropenia. To better describe the characteristics and outcome of ES in adults, a survey was initiated in 2005. The data from 68 patients (60% of them women) fulfilling strict inclusion criteria for ES are reported. The mean age at time of ITP and/or AIHA onset was 52 plus or minus 33 years, both cytopenias occurred simultaneously in 37 cases (54.5%). ES was considered as "primary" in 34 patients (50%) but was associated with an underlying disorder in half of the cases, including mainly systemic lupus, lymphoproliferative disorders, and common variable immunodeficiency. All patients were given corticosteroids, but 50 of them (73%) required at least one "second-line" treatment, including splenectomy(n ‫؍‬ 19) and rituximab (n ‫؍‬ 11). At time of analysis, after a mean follow-up of 4.8 years, only 22 patients (32%) were in remission off treatment; 16 (24%) had died. In elderly patients, the risk of cardiovascular manifestations related to AIHA seems to be higher than the ITP-related risk of severe bleeding. In conclusion, ES is a potentially life-threatening condition that may be associated with other underlying autoimmune or lymphoproliferative disorders. (Blood. 2009;114:3167-3172)

Background: The Caserta and Naples areas in Campania Region experience heavy environmental contamination due to illegal waste disposal and burns, thus representing a valuable setting to develop a general model of human contamination with dioxins (PCDDs-PCDFs) and dioxin-like-PCBs (dl-PCBs). Methods: 94 breastfeeding women (aged 19–32 years; mean age 27.9 ± 3.0) were recruited to determine concentrations of PCDDs-PCDFs and dl-PCBs in their milk. Individual milk samples were collected and analyzed according to standard international procedures. A generalized linear model was used to test potential predictors of pollutant concentration in breast milk: age, exposure to waste fires, cigarette smoking, diet, and residence in high/low risk area (defined at high/low environmental pressure by a specific 2007 WHO report). A Structural Equation Model (SEM) analysis was carried out by taking into account PCDDs-PCDFs and dl-PCBs as endogenous variables and age, waste fires, risk area and smoking as exogenous variables. Results: All milk samples were contaminated by PCDDs-PCDFs (8.6 pg WHO-TEQ/98g fat ± 2.7; range 3.8–19) and dl-PCBs (8.0 pg WHO-TEQ/98g fat ± 3.7; range 2.5–24), with their concentrations being associated with age and exposure to waste fires (p < 0.01). Exposure to fires resulted in larger increases of dioxins concentrations in people living in low risk areas than those from high risk areas (p < 0.01). Conclusions: A diffuse human exposure to persistent organic pollutants was observed in the Caserta and Naples areas. Dioxins concentration in women living in areas classified at low environmental pressure in 2007 WHO report was significantly influenced by exposure to burns.

A predictive model can preoperatively identify patients at risk for needing ABT in lung cancer surgery. The model could be utilized to tailor blood-sparing intervention programs.

The treatment of immune thrombocytopenic purpura (ITP) still offers challenges to clinicians and health professional organizations, despite recommendations provided by international guidelines. In order to improve the care of patients with ITP, it is useful to understand how often such patients receive appropriate treatment and if common errors occur that could be avoided. We retrospectively analyzed all the clinical records between 1 January, 2000 and 31 December, 2002 of patients receiving an ICD-9-CM diagnosis code of 287.3 in three hospitals in northern Italy. We examined whether management strategies of adult men and nonpregnant women with ITP were consistent or not with the guidelines provided by the American Society of Hematology. The ITP diagnosis was confirmed in 120 of 169 patients (71%). Reasons for admission were: medical treatment for ITP (51.7%), medical or surgical treatment of ITP-associated disorders (30.8%), elective splenectomy (15.8%) and diagnosis or observation of ITP (1.7%). Hospital admission resulted appropriate in 78.1% of cases. Therapeutic interventions were appropriate in 100% of cases for glucocorticoid treatment, 86.4% for splenectomy, 47.7% for high-dose immunoglobulins, 40.9% for prophylaxis against bleeding before splenectomy, 33.3% for high-dose glucocorticoid treatment, and 19% for prophylaxis against bacterial infections before splenectomy. Platelet transfusions as treatment for bleeding were appropriate in only 20% of cases. Confirming the usefulness of the American guidelines for ITP, our data suggest that there are important areas of inappropriate management of the disease, which could be corrected by adopting quality improvement programs and studies.

Patients with sarcoidosis are at risk of opportunistic infections both from the sarcoidosis itself and from steroid therapy. Steroid therapy by reducing cell-mediated immunity increases the risk of infection with intracellular organisms. We present a case of Leishmania infection in a 51-year-old Italian woman receiving prednisone for sarcoidosis. The patient lives in Liguria, a region in the northwest of Italy that is considered highly endemic for leishmaniasis. We would postulate that even though leishmaniasis is seen relatively infrequently in connection with sarcoidosis, the occurrence of this opportunistic infection is possible in patients living in areas endemic for such protozoa.