Results 41 to 50 of about 940 (155)

Therapeutic Outcomes in VEXAS Syndrome: A Multicenter Comparative Cohort of Allogeneic Hematopoietic Stem Cell Transplantation and Hypomethylating Agents

American Journal of Hematology, Volume 101, Issue 9, Page 2166-2176, September 2026.
ABSTRACT Hypomethylating agents (HMA) and allogeneic hematopoietic stem cell transplantation (alloHSCT) have both demonstrated remissions in VEXAS; however, comparative data is lacking. We conducted a multicenter, retrospective analysis of 66 patients diagnosed with VEXAS syndrome treated with HMA (n = 35) or alloHSCT (n = 31). Baseline characteristics
Saubia Fathima, Margaret Mei‐Kay Wong, Jesus Gonzalez‐Lugo, Susan M. Geyer, Ali Alsugair, Maria Sirenko, Kimberly J. Langer, Terra L. Lasho, Christy Finke, Jun Choi, Maher Abdul‐Hay, Gary Ho, Mark R. Litzow, Aasiya Matin, Urshila Durani, Mehrdad Hefazi, William J. Hogan, Mithun V. Shah, Aref Al‐Kali, Kebede H. Begna, Naseema Gangat, Antoine N. Saliba, Ronald S. Go, Tariq Kewan, Gabriel Bartoo, Jade Kutzke, Kristen McCullough, Kenneth J. Warrington, Megan Sullivan, Kaaren K. Reichard, Horatiu Olteanu, Hemant Murthy, Talha Badar, Yael Kusne, Jeanne Palmer, Saurabh Chhabra, Nathan Punwani, Mary Riwes, Joseph P. McGuirk, Elizabeth F. Krakow, Amelia Langston, Taxiarchis Kourelis, David Dingli, James Foran, Matthew J. Koster, Mrinal M. Patnaik, David B. Beck, Hassan B. Alkhateeb, Abhishek A. Mangaonkar   +48 more
wiley   +1 more source

Ruxolitinib Pharmacokinetics and Exposure–Toxicity Relationship in Hematologic Malignancies and Immune‐Mediated Diseases: A Prospective Observational Study

Clinical Pharmacology &Therapeutics, Volume 120, Issue 3, Page 753-761, September 2026.
Ruxolitinib pharmacokinetics (PK) has been characterized in clinical trials but remains poorly documented in real‐world practice. This project aimed to investigate ruxolitinib PK in routine clinical practice, identify factors driving its variability, and explore exposure–response relationships to assess the potential role of therapeutic drug monitoring.
Jérémie Tachet, Sara C. Meyer, Francesco Grandoni, Emmanuel Haefliger, Monika Nagy‐Hulliger, Jörg P. Halter, Jakob R. Passweg, David Haefliger, Carine Bardinet, Laurent A. Decosterd, Monia Guidi, François R. Girardin   +11 more
wiley   +1 more source

An Italian cartography of VEXAS‐related thrombosis

HemaSphere, Volume 10, Issue 8, August 2026.
Abstract Thrombotic events (TEs) occur in up to 40% of patients with vacuoles, E1 enzyme, X‐linked, autoinflammatory, and somatic (VEXAS) syndrome, but data on its clinical‐genomics features and anticoagulation strategies are limited. To gain more insight into this, we conducted a two‐step study evaluating the prevalence and outcome of TE in VEXAS ...
Giorgia Ranucci, Maria Rosaria Pascale, Vittorio Forte, Elisa Diral, Corrado Campochiaro, Jacqueline Ferrari, Mariachiara Cuccaro, Chiara Cattaneo, Francesca Crisafulli, Lucia Cardillo, Valeria Renola, Elisa Meddi, Marianna Velocci, Elisa Casciani, Francesca Romano, Pellegrino Musto, Serafina Barbato, Giorgia Battipaglia, Marco Frigeni, Erika Morsia, Arianna Savi, Cristina Papayannidis, Elena Crisà, Laura Cicconi, Isabella Capodanno, Alfonso Fiumarella, Antonio Pierini, Massimo Breccia, Giovanni Orsolini, Giulia Rivoli, Sara Bindoli, Davide Firinu, Antonio Vitale, Bruno Fattizzo, Monica Bocchia, Luca Cantarini, Chiara Pinton, Gabriele Todisco, Nicola Toschi, Paola Triggianese, Adriano Venditti, Maria Teresa Voso, Carmelo Gurnari   +42 more
wiley   +1 more source

Type I and II Interferon Signalling Characterizes the Transcriptional Landscape of Sweet Syndrome

Experimental Dermatology, Volume 35, Issue 7, July 2026.
ABSTRACT Sweet syndrome (SS) is an autoinflammatory neutrophilic dermatosis characterized by abrupt‐onset inflammatory skin lesions and systemic symptoms, yet its molecular pathogenesis remains incompletely defined. To delineate disease‐specific inflammatory programmes, we performed NanoString‐based transcriptomic analysis of SS skin lesions and ...
Laura Calabrese, Chiara Moltrasio, Maurizio Romagnuolo, Pia‐Charlotte Stadler, Zeno Fiocco, Matthias Neulinger‐Muñoz, Rui Aoki, Martina D'Onghia, Pietro Rubegni, Katrin Kerl, Takashi K. Satoh, Angelo V. Marzano, Lars E. French   +12 more
wiley   +1 more source

Case report: Cerebral sinus vein thrombosis in VEXAS syndrome

Frontiers in Medicine
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, and somatic) syndrome is a newly described hemato-inflammatory acquired monogenic entity that presents in adulthood. One of the main features of VEXAS syndrome is a high venous thromboembolism (VTE)
Michael Zisapel, Michael Zisapel, Estelle Seyman, Jeremy Molad, Jeremy Molad, Hen Hallevi, Hen Hallevi, Michal Mauda-Havakuk, Michal Mauda-Havakuk, Tali Jonas-Kimchi, Tali Jonas-Kimchi, Ori Elkayam, Ori Elkayam, Tali Eviatar, Tali Eviatar   +14 more
doaj   +1 more source

Australian clinical practice guideline: diagnosis and treatment of idiopathic multicentric Castleman disease

Internal Medicine Journal, Volume 56, Issue 7, Page 1218-1239, July 2026.
Abstract Idiopathic multicentric Castleman disease (iMCD) is a rare condition. The pathogenesis is incompletely understood; however, interleukin‐6 (IL‐6) is a major mediator. The clinical presentation is heterogeneous, from mild constitutional symptoms to severe multi‐organ failure.
Dipti Talaulikar, Jun Yen Ng, Adam Bryant, Annmarie Bosco, Hugh C. Caterson, Pratyush Giri, Pravin Hissaria, Simon J. Harrison, Stephen Lade, Connull Leslie, Jacinta Perram, H. Miles Prince, Dejan Radeski, Maureen Rischmueller, Craig Wallington‐Gates, Nicholas Weber, Alberta Hoi   +16 more
wiley   +1 more source

Performance Characteristics of Anti–Collagen II Antibodies in Relapsing Polychondritis and Related Diseases: Prospective Analysis, Systematic Review, and Meta‐Analysis

Arthritis Care &Research, Volume 78, Issue 6, Page 798-809, June 2026.
Objective Relapsing polychondritis (RP) is a rare disease defined by recurrent cartilaginous inflammation. Anti–collagen II (Col2) antibodies have been proposed as a diagnostic biomarker for RP, but their performance characteristics are not well defined.
Karyssa Stonick, Marcela A. Ferrada, Alice Fike, Kaitlin A. Quinn, Benjamin A. Turturice, Casey Stein, Peter C. Grayson   +6 more
wiley   +1 more source

A Case of VEXAS (Vacuoles, E1 Enzyme, X-Linked, Autoinflammatory, Somatic) Syndrome With Cardiac Involvement

Annals of Internal Medicine: Clinical Cases
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome was first described in late 2020. It occurs as the result of somatic mutations in the ubiquitin-activating enzyme UBA1.
Xiaocao Xu, Najiya Haque, Chiamaka Onah, Lauren Dudley   +3 more
doaj   +1 more source

Current status and prospects of diagnosis and treatment of VEXAS syndrome [PDF]

Zhenduanxue lilun yu shijian
VEXAS (Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently recognized autoinflammatory disease involving multiple systems, caused by somatic mutations in the ubiquitin-like modifier activating enzyme 1 (UBA1) gene.
QIAN Haozhou, CHANG Chunkang
doaj   +1 more source

A Novel Use of Anifrolumab for a Novel Disease

ACR Open Rheumatology, Volume 8, Issue 5, May 2026.
Objective To explore the potential role of type‐I interferon (IFN‐I) signaling in autoimmune cytopenias outside the context of systemic lupus erythematosus (SLE), and to evaluate the clinical response to off‐label anifrolumab in two patients with fatigue and immune‐mediated cytopenias who did not meet established criteria for SLE.
Zachary Holtz, Adam Schulz, Alexa Meara
wiley   +1 more source