Multiple Tumors in a Patient with Interleukin-2-Inducible T-Cell Kinase Deficiency: A Case Report.

Immune dysregulation in Inborn Errors of Immunity (IEI) shows a broad phenotype, including autoimmune disorders, benign lymphoproliferation, and malignancies, driven by an increasing number of implicated genes. Recent findings suggest that childhood cancer survivors (CCSs) may exhibit immunological abnormalities potentially linked to an underlying IEI, along with a well-known increased risk of subsequent malignancies due to prior cancer treatments. We describe a patient with two composite heterozygous pathogenic variants in the interleukin-2-inducible T-cell kinase () gene and a history of multiple tumors, including recurrent Epstein-Barr virus (EBV)-related nodular sclerosis and Hodgkin's lymphoma (NSHL), associated with unresponsive multiple hand warts, immune thrombocytopenia, and an impaired immunological profile (CD4+ lymphocytopenia, memory B-cell deficiency, reduction in regulatory T-cells, and B-cell- and T-cell-activated profiles).

A 28 years surgical experience of 398 sinusal mucoceles: how technologies, techniques and experiences have changed their treatment throughout the years.

Purpose: Sinonasal mucoceles (SM) are benign, expansive neoformations which require surgical marsupialization to prevent severe complications. The present study reports the larger case history ever published: a 28-years expertise in the surgical management of SM.

Methods: A retrospective study was conducted on patients surgically treated for SM at the Departments of Otorhinolaryngology of Varese and Pavia, between 1994 and 2022.

Late-onset and long-lasting neutropenias in the young: A new entity anticipating immune-dysregulation disorders.

This study identifies a new chronic form of immune neutropenia in the young with or without detectable indirect anti-neutrophil antibodies, characterized by mild/moderate neutropenia low risk of severe infection (14%), tendency to develop autoimmune phenomena over the course of the disease (cumulative incidence of 58.6% after 20 years of disease duration), leukopenia, progressive reduction of absolute lymphocyte count and a T- and B-cell profile similar to autoimmune disorders like Sjogren syndrome, rheumatoid arthritis, and systemic lupus erythematosus (increased HLADR+ and CD3 + TCRγδ cells, reduced T regulatory cells, increased double-negative B and a tendency to reduced B memory cells). In a minority of patients, P/LP variants related to primary immuno-regulatory disorders were found.

Open-Source Image-Based Tool to Experimentally Evaluate Blood Residence Time in Clinical Devices.

This article introduces an open-source tool to experimentally compare blood residence time in biomedical devices using an image-based method. The experimental setup and the postprocessing workflow are comprehensively elucidated in a detailed report that conducts a thorough comparison of the residence times of a blood analog within three distinct blood oxygenator prototypes. To enable widespread accessibility and ease of use, the user-friendly MATLAB App developed for the analysis is available on the Mathworks repository: https://www.