Publications by authors named "Irene Defrancesco"

The coronavirus disease 2019 (COVID-19) pandemic created major challenges for allogeneic hematopoietic stem cell transplantation (allo-HSCT). Scientific societies and authorities recommended cryopreserving grafts before starting conditioning regimens, despite limited data on the clinical impact. The Italian Group for Bone Marrow Transplantation (GITMO) conducted a registry-based study involving 3492 patients who underwent allo-HSCT between March 2018 and September 2021.

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Background: Infection by SARS-CoV2 has become a challenge, especially for immunocompromised patients who show a weaker humoral response to COVID-19 vaccine. Tixagevimab+cilgavimab (Evusheld) is a combination of human monoclonal antibodies that can be used for pre-exposure prophylaxis to prevent infection or disease by SARS-CoV2.

Objectives: Our study aimed to investigate the effectiveness of Evusheld by comparing an Exposed and an Unexposed group.

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Hepatitis C virus (HCV)-associated diffuse large B-cell lymphoma (DLBCL) displays peculiar clinicopathological characteristics, but its molecular landscape is not fully elucidated. In this study, we investigated the clinicopathological and molecular features of 54 patients with HCV-associated DLBCL. The median age was 71 years.

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  • Eosinophilic dermatosis of hematologic malignancy (EDHM) is believed to be caused by immune dysregulation rather than just insect bites, but its overall impact on prognosis remains uncertain.
  • A study evaluated 15 oncohematological patients diagnosed with EDHM, revealing varied skin lesions and a connection to chemotherapy treatments and potential insect bites.
  • Histopathological similarities to insect bites were noted, suggesting a link between EDHM and more aggressive forms of hematologic disease.
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  • * Out of 12 enrolled patients, 10 responded well while 2 did not; 58.3% experienced adverse reactions, highlighting the complexities in treatment response and side effects.
  • * While the overall diversity of GM did not significantly differ between groups, there were notable differences in specific bacterial taxa, suggesting that further research is needed to explore these relationships.
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  • MYD88 is a key biomarker for predicting therapy response in IgM monoclonal gammopathies, typically detected using allele-specific quantitative PCR (ASqPCR) or the newer droplet digital PCR (ddPCR).
  • A study compared these two methods and found a 74% agreement in results, with ddPCR showing superior sensitivity, especially for bone marrow samples and low mutational burden samples like peripheral blood and cfDNA.
  • The findings indicate that ddPCR is a promising method for MYD88 detection across various sample types, and there are distinct mutational differences between patients with Waldenström Macroglobulinemia and those with IgM monoclonal gammopathy that warrant further exploration.
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Chimeric Antigen Receptor-T cells (CAR-T) are considered novel biological agents, designed to selectively attack cancer cells expressing specific antigens, with demonstrated clinical activity in patients affected with relapsed/refractory B-cell malignancies. In consideration of their complexity, the use of CAR-T requires dedicated clinical setting and health care practitioners with expertise in the selection, treatment, and management of toxicities and side effects. Such issue appears particularly important when contextualized in the rapid progress of CAR-T cell treatment, translating into a constant need of updating and evolution.

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  • Non-chronic lymphocytic leukemia (non-CLL) clonal B-cell lymphocytosis (CBL) is a complex group of blood disorders that remain poorly understood, necessitating further research.
  • A study of 28 patients revealed significant genetic diversity, with several key mutations found, including MYD88 and others, indicating that various pathways may contribute to these disorders rather than aligning with existing lymphoma categories.
  • The findings provide insight into the molecular characteristics of non-CLL CBL but highlight the need for improved diagnosis, prognosis, and treatment approaches in clinical settings.
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  • * This study evaluated 11 patients with relapsed or refractory HCV-positive DLBCL who received DAAs, either alongside or after salvage chemotherapy, primarily using sofosbuvir-based regimens.
  • * All patients completed their DAA courses and achieved a sustained virological response without serious side effects; the overall survival rate after four years was 76%, supporting the use of DAAs in similar cases.
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Hepatic veno-occlusive disease (VOD) or sinusoidal obstruction syndrome (SOS) is a rare complication characterized by hepatomegaly, right-upper quadrant pain, jaundice, and ascites, occurring after high-dose chemotherapy, hematopoietic stem cell transplantation (HSCT) and, less commonly, other conditions. We review pathogenesis, clinical appearance and diagnostic criteria, risk factors, prophylaxis, and treatment of the VOD occurring post-HSCT. The injury of the sinusoidal endothelial cells with loss of wall integrity and sinusoidal obstruction is the basis of development of postsinusoidal portal hypertension responsible for clinical syndrome.

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While classical nodal mantle cell lymphoma (cMCL) is often associated with involvement of multiple extranodal sites, isolated extranodal disease (ED) at the time of diagnosis is a rare event; data on the outcome of these forms are lacking. On behalf of the European MCL Network, we conducted a retrospective analysis on the clinical characteristics and outcomes of MCL presenting with isolated or predominant ED (MALT MCL). We collected data on 127 patients with MALT MCL diagnosed from 1998 to 2015: 78 patients (61%) were male with a median age of 65 years.

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Hepatitis C virus infection represents a global health problem with 3% of population infected worldwide. Several epidemiological studies have shown an increased risk of B cell non-Hodgkin lymphomas in HCV-infected subjects with a wide geographic variability. The observation that HCV eradication by antiviral treatment is associated with successful lymphoma response provided the most convincing evidence for the causal role of HCV in lymphoma's development.

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Marginal zone lymphomas (MZLs) are indolent nonfollicular B-cell lymphomas (INFLs) and have heterogeneous clinical behavior. Recently, time to progression of disease at 24 months (POD24) was identified to stratify overall survival (OS) in follicular non-Hodgkin lymphoma and in INFL. Here, we examined the ability of POD24 to predict subsequent OS in a large, international cohort of MZL as part of the NF10 prospective international registry headed by Fondazione Italiana Linfomi (FIL).

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IgM monoclonal gammopathies of undetermined significance (IgM MGUS) are associated with a risk of progression to Waldenström macroglobulinaemia (WM) or other lymphoproliferative disorders (LPD) of 1-2% per year. We analysed 176 consecutive patients with IgM MGUS to evaluate risk factors for progression. With a median follow-up of 83 months (1214 person-years), 15 patients (8·5%) progressed to WM (n = 14) or marginal zone lymphoma (n = 1).

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Chronic hepatitis C virus (HCV) infection is related with an increased risk of non-Hodgkin lymphomas (NHL). In indolent subtypes, regression of NHL was reported after HCV eradication with antiviral therapy (AT). In 2008 in Lombardy, a region of Northern Italy, the "Rete Ematologica Lombarda" (REL, Hematology Network of Lombardy-Lymphoma Workgroup) started a prospective multicenter observational cohort study on NHL associated with HCV infection, named "Registro Lombardo dei Linfomi HCV-positivi" ("Lombardy Registry of HCV-associated non-Hodgkin lymphomas").

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Extranodal marginal zone B-cell lymphomas (EMZLs) of the mucosa-associated lymphoid tissue (MALT) are indolent lymphomas which can present at any extranodal site. The most frequent localizations (other than stomach) are ocular adnexa, salivary gland, skin, lung and thyroid. Chronic inflammation and antigenic stimulation are a potential risk for the development of MALT lymphomas.

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The Bing-Neel syndrome is a rare neurological complication of Waldenström's Macroglobulinemia which results from a direct involvement of central nervous system by malignant lymphoplasmacytic cells. The clinical suspicion of Bing-Neel syndrome may be overlooked because neurologic symptoms are heterogeneous, nonspecific and sometimes underhand. A definitive diagnosis of Bing-Neel syndrome can be confidently made using brain and spinal cord magnetic resonance imaging as well as histopathology and/or cerebrospinal fluid analysis to confirm the neoplastic infiltration of central nervous system.

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