Hematopoietic Stem Cell Transplantation in Thalassemia and Sickle Cell Disease. Unicenter Experience in a Multi-Ethnic Population.

Hematopoietic stem cell transplantation (HSCT) still remains the only definitive cure currently available for patients with thalassemia and sickle cell anemia. Results of transplant in thalassemia and in sickle cell anemia have steadily improved over the last two decades due to improvements in preventive strategies, and effective control of transplant-related complications. From 2004 through 2009, 145 consecutive patients with thalassemia and sickle cell anemia, ethnically heterogeneous from Mediterranean and Middle East countries, were given HSCT in the International Center for Transplantation in Thalassemia and Sickle Cella Anemia in Rome. This experience is characterized by two peculiarities: patients were ethnically very heterogeneous and the vast majority of these patients were not regularly transfesed/chelated and therefore were highly sensitized due to RBC transfusions without leukodepletion filters. Consequently, they could have a high risk of graft rejection as a result of sensitization to HLA antigens. The Rome experience of SCT in patients with thalassemia and sickle cell anemia confirmed the results obtained in Pesaro, and most importantly showed the reproducibility of these results in other centers

Is it possible to study paleoenvironmental changes in Alpine spring habitats? A few examples from the south-eastern Alps (NE Italy)

Palaeolimnological techniques were applied to spring habitats in the Trentino Region (NE Italy) as a test in order to reconstruct past environmental changes in these crucial and fragile milieus. Three different sites were selected from a database of more than one hundred on the basis of morphological, geological, and biological factors, including human impact evaluation: Nambrone, Paul and Madonnina Val Lomasona. Sampling was performed by adapting standard lake-sediment coring methods, and the retrieved sediment was described and subsampled following standard "lake" procedures. Field work and sedimentological analyses revealed clear influence from nearby fluvial environment for the Nambrone site, whereas Paul and Madonnina Val Lomasona were selected for further analyses (e.g., dating, diatoms, chironomids). Lead-210 and Caesium-137 dating methods were used to obtain a chronological framework, in addition to historical information on land use and other events in the area. The upper part of the Paul sequence presented organic rich sediment compatible with spring environments, but the lower and essentially detrital part appeared to record alluvial deposition, probably linked to extraordinary flood events (1882, 1966). This interpretation is indirectly confirmed by the records of the fallout nuclides Caesium-137 and Lead-210, that were both unfortunately concentrated in the topmost 1 cm, making it impossible to construct a normal age-depth profile. Madonnina Val Lomasona sedimentological and biological indicators point to a marked change around ca 5 cm, dividing the sequence in two units, corresponding to different environmental conditions. Although the interpretation of the dating results is not completely straightforward, the assembled data suggest that the upper 5 cm of the record represents ca the last 50 years. During this period we found typical limnocrenic, clear-water spring conditions, while before 1960s the coexistence of lotic and lentic chironomid species including several opportunistic ones and the low richness, emphasized the presence of a disturbance (e.g., highly variable flow and/or trophic conditions), as confirmed also by diatom assemblages and sedimentological features. The recorded disturbance is probably linked to a water-level change due to the local land use, as also testified by historical data. The results of this study support the idea that carefully selected spring sites might be approached with adapted palaeolimnological techniques to extract valuable palaeoenvironmental information. However, it also highlighted the low percentage of "good sites" (1%) and considerable difficulties in coring and sampling without disturbing the sediment

COVID-19 pneumonia and pulmonary microembolism in a patient with B-thalassemia major

We think that thalassemia is not necessarily a cause of aggravation of the clinical course in COVID-19; however, certain key factors must be considered, such as the anemic condition, the likely pathogenic role of the virus on hemoglobin, and the hypercoagulable state to prevent any complications

A Gis-based cost distance approach to analyse the spread of Matsucoccus feytaudi in Tuscany, Italy (Coccoidea Matsucoccidae)

The Maritime pine bast scale Matsucoccus feytaudi Ducasse was introduced into south-eastern France from the Atlantic regions and since then has moved eastward to reach Tuscany, Italy, causing decay and death of thousands of hectares of pine stands. Monitoring of the pine bast scale by pheromone traps in Tuscany since 2000 has allowed us to obtain yearly data on the pest’s diffusion. The data were processed by means of the Thiessen polygons algorithm to obtain the distribution of the insect. A GIS cost distance model was adopted to simulate the spread of M. feytaudi infestation: dominant winds were used in the least accumulative distance calculation, together with Pinus pinaster presence as secondary factor. The overall accuracy of the model calculated for the years 2003 to 2008 ranged between 70.0% and 81.8%; user’s and producer’s accuracy showed greater variability, but with good performances in the majority of cases. The use of pheromone traps and spatial analysis enabled to produce annual maps for the Tuscany, with the identification of the Maritime pine forests more susceptible to M. feytaudi colonization. This cartography was an important tool for regional offices in the identification of areas requiring direct forestry management

Bone Marrow Clonogenic Capability, Cytokine Production, and Thymic Output in Patients with Common Variable Immunodeficiency

AbstractIn patients with primary Ab deficiencies, hematological and immunological abnormalities are frequently observed. A regenerative failure of hemopoietic stem/progenitor cells has been hypothesized. We evaluated in the bone marrow (BM) of 11 patients with common variable immunodeficiency, the phenotype of BM progenitors and their in vitro growth by colony-forming cell (CFC) and long-term culture (LTC) assays. A significant decrease in erythroid and mixed CFC and, to a greater extent, in primitive LTC-CFC progenitors was observed in patients compared with healthy controls. The frequency of BM pre-B and pro-B cells correlated directly with the absolute number of CD19+ lymphocytes. BM cells cultured in vitro produced spontaneously lower amounts of IL-2 and elevated levels of TNF-α compared with controls, indicating a skewing toward a proapoptotic cytokine pattern. In addition, stromal cells generated after BM LTC secreted less IL-7 and displayed by immunohistochemistry an altered phenotype. These findings were associated with a significant decrease in naive Th cells coexpressing CD31 in the peripheral blood. These results indicate an impaired growth and differentiation capacity of progenitor cells in patients with common variable immunodeficiency

Modulation of Human Immunodeficiency Virus 1 Replication by Interferon Regulatory Factors

Transcription of the human immunodeficiency virus (HIV)-1 is controlled by the cooperation of virally encoded and host regulatory proteins. The Tat protein is essential for viral replication, however, expression of Tat after virus entry requires HIV-1 promoter activation. A sequence in the 5′ HIV-1 LTR, containing a binding site for transcription factors of the interferon regulatory factors (IRF) family has been suggested to be critical for HIV-1 transcription and replication. Here we show that IRF-1 activates HIV-1 LTR transcription in a dose-dependent fashion and in the absence of Tat. This has biological significance since IRF-1 is produced early upon virus entry, both in cell lines and in primary CD4+ T cells, and before expression of Tat. IRF-1 also cooperates with Tat in amplifying virus gene transcription and replication. This cooperation depends upon a physical interaction that is blocked by overexpression of IRF-8, the natural repressor of IRF-1, and, in turn is released by overexpression of IRF-1. These data suggest a key role of IRF-1 in the early phase of viral replication and/or during viral reactivation from latency, when viral transactivators are absent or present at very low levels, and suggest that the interplay between IRF-1 and IRF-8 may play a key role in virus latency

Indici per la valutazione della qualit? ecologica dei laghi

Methods are presented to evaluate the biological quality of Italian lakes based on phytoplankton, macrophytes, fishes, benthic fauna and hydromorphology

The use of alteplase, although safe, does not offer clear clinical advantages when mild stroke is non-disabling

IntroductionIt is unknown whether alteplase is effective and safe in patients with mild acute ischemic stroke (AIS). Determining whether symptoms are “disabling” or not is a crucial factor in the management of these patients. This study aimed to investigate the efficacy and safety of alteplase in patients with mild, non-disabling AIS.MethodsWe included all consecutive patients admitted for AIS at our institution from January 2015 to May 2022 who presented a baseline NIHSS score of 0–5 and fit the criteria to receive intravenous thrombolysis. In order to select only subjects with non-disabling AIS, we excluded patients who scored more than 1 point in the following NIHSS single items: vision, language, neglect, and single limb. Patients who scored at least 1 point in the NIHSS consciousness item were excluded as well. This study is a retrospective analysis of a prospectively collected database.ResultsAfter the application of the exclusion criteria, we included 319 patients, stratified into patients receiving and not receiving alteplase based on non-disabling symptoms. The two groups were comparable regarding demographic and clinical data. Rates of a 3-month favorable outcome, defined as a 3-month mRS score of 0–1, were similar, being 82.3% and 86.1% in the treated and untreated patients, respectively. Hemorrhagic complications and mortality occurred infrequently and were not affected by alteplase treatment.DiscussionThis observational study suggests that the use of alteplase, although safe, is not associated with a better outcome in highly selected patients with non-disabling AIS