Internal medicine
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Review
Clinical Challenges of Emerging Acquired Autoinflammatory Diseases, Including VEXAS Syndrome.
Vacuoles, E1-ubiquitin-activating enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome, caused by an acquired mutation in the ubiquitin-activating enzyme ubiquitin-like modifier activating enzyme 1 (UBA1), was discovered in 2020. Since then, many cases have been reported worldwide. Recently, we performed UBA1 genetic testing in suspected cases of VEXAS throughout Japan and investigated the clinical features of these cases. ⋯ As the concept of "acquired autoinflammatory diseases," including VEXAS syndrome, has gained popularity, the number of suspected cases is expected to increase. Currently, there are no established diagnostic or treatment guidelines for these conditions, and they need to be urgently developed. This review summarizes the clinical problems faced by patients with acquired autoinflammatory diseases, including VEXAS.
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Objective Pembrolizumab plus platinum and pemetrexed (Pemb-Plt-PEM) combination therapy is an effective first-line treatment for advanced non-squamous non-small-cell lung cancer (NSCLC), regardless of programmed death-ligand 1 expression. However, the effectiveness and feasibility of first-line Pemb-Plt-PEM therapy in elderly patients (≥75 years old) remain unclear. Therefore, this study investigated the safety and efficacy of first-line Pemb-Plt-PEM in elderly patients with non-squamous NSCLC. ⋯ Furthermore, the sex and NLR influenced the association between Pemb-Plt-PEM and the OS. The OS for low and high NLR values was 32.8 and 2.6 months, respectively. Conclusion First-line Pemb-Plt-PEM therapy is effective and feasible in elderly patients with non-squamous NSCLC.
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Case Reports
Successful Treatment of Methotrexate-associated Lymphoproliferative Disorder with the Pola-R-CHP Regimen.
Methotrexate-associated lymphoproliferative disorder (MTX-LPD) constitutes a subset of lymphoid proliferations and lymphomas that are associated with immune deficiency and dysregulation. The clinical management of MTX-LPDs is contingent on their histopathological subtypes. ⋯ We recently encountered a case of DLBCL-type MTX-LPD with parathyroid hormone-related protein-C (PTHrP)-mediated hypercalcemia that was managed with polatuzumab vedotin, rituximab, cyclophosphamide, doxorubicin, and prednisone (Pola-R-CHP). We herein report our experience to encourage hematologists to explore the safe and effective use of Pola-R-CHP under such conditions.