Objective To identify patients with VEXAS syndrome (vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic syndrome) from a single‐center cohort of Italian patients with vasculitis, using a clinically oriented phenotype‐first approach. Methods We retrospectively reviewed the clinical records of 147 consecutive male patients followed up in our vasculitis clinic from 2013 to date. All patients with a diagnosis of vasculitis and treatment‐resistant manifestations of inflammation, persistently elevated inflammation markers, and hematologic abnormalities were identified. Bone marrow aspirates were examined for the presence of vacuoles. Sequencing of ubiquitin‐activating enzyme E1 (UBA‐1) was performed using genomic DNA from peripheral blood leukocytes or bone marrow tissue. Results Seven patients with vasculitis and concomitant features of VEXAS syndrome were identified. A final diagnosis of VEXAS syndrome was made in 3 of the 5 patients who underwent sequencing of UBA‐1 (diagnosis was made postmortem for 1 patient). In all 3 patients, examination of the bone marrow aspirate revealed vacuoles characteristic of VEXAS syndrome, and all 3 patients met the definitive World Health Organization criteria for myelodysplastic syndrome. Cytogenetic analysis showed normal karyotypes in all 3 patients. Conclusion To our knowledge, this is the first report of VEXAS syndrome associated with antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis. Our data emphasize the need to consider VEXAS syndrome when evaluating patients with various forms of systemic vasculitis. The novel association between VEXAS syndrome and ANCA‐associated vasculitis reported herein warrants further investigation.

Considering the transversal unmet need for the counselling of advanced cancer patients treated with immune checkpoint inhibitors (CKI) about influenza vaccination, we planned an explorative study to assess the efficacy of influenza vaccine in this population, its potential impact on the severity and mortality of influenza syndrome (IS) and on of anticancer immunotherapy outcome. METHODS: INVIDIa was a retrospective, observational, multicenter, explorative study at 21 Italian centers, enrolling consecutive advanced cancer patients receiving treatment with CKI during the Italian influenza vaccinal season 2016-2017. All data regarding influenza vaccination, IS and treatment with CKI were collected. Subgroup analyses were pre-planned for elderly and lung cancer patients. RESULTS: The study enrolled 300 patients, 79 of which received flu vaccine; 221 unvaccinated patients constituted the control group. The incidence of IS was of 24.1% among vaccinated, vs 11.8% of controls; OR=2.4 (95%CI=1.23-4.59; p=0.009). Severity of

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Background: Cancer immunotherapy directs the immune system to attack tumor cells by targeting tumor-associated antigens. We manufacture fully personalized monocytederived dendritic cell-based vaccines (DC-based vaccines) that are evaluated in investigator-initiated clinical trial "Combined antitumor therapy with ex vivo manipulated dendritic cells producing interleukin-12 in children, adolescents and young adults with progressive, recurrent or primarily metastatic high-risk tumors". Methods: DC-based vaccine, called MyDendrix, is manufactured under GMP in Clean rooms of the Department of Pharmacology Masaryk University Brno. Mononuclear cells are collected by apheresis. Monocytes cultivated with IL-4 and GM-CSF differentiate into DC and are exposed to autologous tumor lysate antigens. Semi-maturated DC are aliquoted and cryopreserved. Quality control includes viability, IL-12, IL-10 production, surface markers expression, stimulation of allogenic and autologous T-cells. Vaccines are administered intradermally every 2-4 weeks, up to 35 doses. Detailed immunomonitoring of peripheral blood leucocytes subsets at the baseline and during vaccination is performed. That includes routine examined peripheral blood parameters, monocyte subsets, MDSC, specific lymphocyte subsets including Tregs, effector Tcells, NKT-like cells. Moreover, ex vivo functional immunomonitoring based on autologous T-cell stimulation by DC vaccine is performed. Results: The primary endpoint of the clinical trial is the assessment of safety by the analysis of occurrence of AESI (adverse events of special interest) in adaptive 5 þ 5þ5 þ 5 design. As of to date, 11 patients have been treated. No AESI was detected. Local skin reaction is usually mild and self-limiting. De facto size and redness of induration at vaccination sites can be reflected by T-cells stimulation after vaccination. Ex vivo T-cell reactivity was enhanced upon vaccination. Conclusions: Intradermal treatment with the DC-based anti-cancer vaccine is, according to interim analysis, safe and well-tolerated. Ex vivo assessment of pre-and post-vaccination DC-stimulated autologous T-cell activation has shown that anti-cancer vaccine-enhanced pre-existing T-cell response to tumor antigens.