Clinical & Experimental Immunology, Volume 206, Issue 3, December 2021 ..read more

Abstract Innate and adaptive immune responses in the central nervous system (CNS) play critical roles in the pathogenesis of neurological diseases. In the first of a two-part special issue, leading researchers discuss how imaging modalities are used to monitor immune responses in several neurodegenerative diseases and glioblastoma and brain metastases. While comparative studies in humans between imaging and pathology are biased towards the end stage of disease, animal models can inform regarding how immune responses change with disease progression and as a result of treatment regimens. Magneti ..read more

Clinical & Experimental Immunology, Volume 206, Issue 2, November 2021 ..read more

Abstract Since the first studies of the nervous system by the Nobel laureates Camillo Golgi and Santiago Ramon y Cajal using simple dyes and conventional light microscopes, microscopy has come a long way – to the most recent techniques that make it possible to perform images in live cells and animals, in health and disease. Many pathological conditions of the central nervous system have already been linked to inflammatory responses. In this scenario, several available markers and techniques can help imaging and unveil the neuroinflammatory process. Moreover, microscopy imaging techniques have ..read more

Summary Brain tumors and brain metastases induce changes in brain tissue remodelling that lead to immunosuppression and trigger an inflammatory response within the tumor microenvironment. These immune and inflammatory changes can influence invasion and metastasis. Other neuroinflammatory and necrotic lesions may occur in patients with brain cancer or brain metastases as sequelae from treatment with radiotherapy. Glioblastoma (GBM) is the most aggressive primary malignant brain cancer in adults. Imaging methods such as positron-emission tomography (PET) and different magnetic resonance imaging ..read more

Abstract Acquired angioedema due to C1-inhibitor (C1-INH) deficiency (AAE-C1-INH) is rare and is associated with underlying lymphoproliferative diseases. C1-INH deficiency may be due to neoplastic overconsumption of C1-INH and the generation of anti-C1-INH autoantibodies. Uncovering an occult malignancy can lead to earlier oncology referral and improvement of angioedema after treatment of the underlying lymphoproliferative disorder. We characterized 7 patients with C1-INH-AAE that highlight the importance of recognizing the association between C1-INH-AAE and underlying malignancy. In acute att ..read more

Abstract This large cohort study from the US Premier Healthcare Database evaluated the risk and predictors of anaphylaxis in association with intravenous immunoglobulin (IVIg) therapy in the inpatient and outpatient setting. Data were collected retrospectively (01/2009-12/2018) from 24,919 patients administered IgPro10 IVIg, median age 54 years. Immunoglobulins of interest were IgPro10 and other IVIg given before or after IgPro10. Moderate and severe anaphylaxis was identified from same-day parenteral epinephrine and IVIg use and reviews of patient record summaries. Predictors for first anaphy ..read more

Abstract Hepatocellular carcinoma (HCC) is the world’s leading cause of tumor-related mortalities. Natural killer (NK) cells play a critical role at the first immunological defense line against HCC initiation and progression. NK cell dysfunction is therefore an important mechanism for immune evasion of HCC cells. In the present study using a murine HCC model, we revealed the down-regulation of PR/SET Domain 10 (PRDM10) in hepatic NK cells that were phenotypically and functionally exhausted. PRDM10 silencing diminished the expression of natural killer group 2 member D (NKG2D) and tumor necrosis ..read more

Abstract Behçet's syndrome (BS) is a systemic vasculitis with several clinical manifestations. Neutrophil hyperactivation mediates vascular BS pathogenesis, via both a massive reactive oxygen species (ROS) production and neutrophil extracellular traps (NETs) release. Here, we investigated neutrophil-mediated mechanisms of damage in non-vascular BS manifestations and explored the in vitro effects of colchicine in counteracting these mechanisms. NETs and intracellular ROS production was assessed in blood samples from 80 BS patients (46 with active non-vascular BS, 34 with inactive disease) and 8 ..read more

Abstract MALT1-deficiency is a rare combined immunodeficiency characterized by recurrent infections, dermatitis and enteropathy. We herein investigate the immunological profiles of our patient and previously reported children with MALT1-deficiency. A mutation analysis was performed by targeted panel sequencing for primary immunodeficiency. Lymphocyte subset, activation and B cell differentiation were analyzed by flow-cytometry and t-Distributed Stochastic Neighbor Embedding. Pneumocystis pneumonia developed in a 6-month-old Japanese infant with atopic dermatitis, enteritis and growth restricti ..read more