Results 71 to 80 of about 1,455 (196)

Hypomethylating agents in vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic syndrome (VEXAS): A systematic review

British Journal of Haematology, Volume 208, Issue 3, Page 819-828, March 2026.
Summary VEXAS syndrome (vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic) is an X‐linked, systemic, haemato‐inflammatory syndrome caused by somatic mutations in the UBA1 gene. No standardized treatment guidelines exist, but evidence is emerging that treatment with hypomethylating agents (HMAs) can induce improvement of the inflammatory symptoms,
Fieke W. Hoff, Roochi Trikha, Emma M. Groarke, Bhavisha A. Patel   +3 more
wiley   +1 more source

Schnitzler Syndrome as an Autoinflammatory Disease Driven by B‐Cell‐Specific Somatic MYD88 Mutation

Allergy, EarlyView.
Yuyi Zhou, Yoko Ueki, Naoya Iwata, Hiroshi Oue, Kentaro Kato, Mengyan Li, Kazushi Izawa, Tomoyasu Jo, Seishi Ogawa, Kenji Kabashima, Naotomo Kambe   +10 more
wiley   +1 more source

Distinct Pattern of Atypical Megakaryocytes in VEXAS Syndrome

International Journal of Laboratory Hematology, EarlyView.
Andrew Y. Sung, Jackson W. Douglas, Olga K. Weinberg, Sharon K. Germans, Weina Chen   +4 more
wiley   +1 more source

Serum Cytokine Profiling Differentiates Underlying Diseases in Cytokine Storm Syndrome

Arthritis &Rheumatology, Volume 78, Issue 2, Page 463-474, February 2026.
Serum cytokine profiling was performed using Luminex (48 cytokines) and ELISA (5 cytokines). Assessment of key cytokines (IFN‐α, IL‐18, IL‐6, CXCL9) delineated five dominant inflammatory patterns: I, IFN‐α–dominant CSS; II, IL‐18–dominant CSS; III, IL‐6–dominant CSS; IV, IFN‐γ–dominant CSS; and V, IL‐6 & IFN‐γ intermediate CSS.
Shuya Kaneko, Maho Hatano, Asami Shimbo, Futaba Miyaoka, Hitoshi Irabu, Yuko Akutsu, Yuko Hayashi, Mao Mizuta, Yasuo Nakagishi, Keiji Akamine, Naomi Iwata, Kenji Furuno, Takayuki Tanaka, Kazuyuki Ueno, Shuhei Fujita, Koji Yokoyama, Toshinori Minato, Kazushi Izawa, Takahiro Yasumi, Tadashi Matsubayashi, Tadashi Hosoya, Hiroki Nishikawa, Junya Fujimura, Ryoko Asano, Yuko Sugita, Kenichi Watanabe, Anna Kobayashi, Takuya Endo, Katsuhide Eguchi, Ryuta Nishikomori, Ryuhei Yasuoka, Takaki Asano, Miyako Kanno, Kazuya Hamada, Yuji Fujita, Daisuke Hayashi, Shojiro Watanabe, Takeshi Shiba, Shinsuke Yasuda, Masaaki Mori, Hirokazu Kanegane, Masatoshi Takagi, Masaki Shimizu   +42 more
wiley   +1 more source

Prognostic value of flow cytometry in myelodysplastic neoplasms (MDS): Composition of a FCM‐prognostic score (FCM‐PS) for overall survival

HemaSphere, Volume 10, Issue 2, February 2026.
Abstract Flow cytometry (FCM) is a co‐criterion in myelodysplastic neoplasms (MDS) diagnostics, currently not used for prognostication. This study aimed to develop an FCM‐score predicting overall survival (OS) in MDS to improve early clinical patient prognostication.
Aida Santaolalla, Uta Oelschlaegel, Susann Winter, Shirin Jamshidi, Theresia M. Westers, Katja Sockel, Martin Bornhäuser, Rosa Andres Ejarque, Farzin Farzaneh, Antonella Poloni, Anne‐Sophie Kubasch, Mieke Van Hemelrijck, Arjan A. van de Loosdrecht, Uwe Platzbecker, Shahram Kordasti   +14 more
wiley   +1 more source

VEXAS syndrome as a cause for multifocal, relapsing head and neck inflammation

Clinical Case Reports
Key Clinical Message VEXAS syndrome (vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic) is a novel autoinflammatory syndrome. We describe a case of VEXAS syndrome with upper airway and oral cavity involvement which are not well described in the ...
Aoife Heeney, Rachael Wu, Conall Fitzgerald, Nina Orfali, Nadim Akasheh, Conor Magee   +5 more
doaj   +1 more source

Clinical Images: Vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic syndrome presenting as aseptic pustular dermatosis: A rare clinical manifestation

Arthritis &Rheumatology, Volume 78, Issue 3, Page 763-763, March 2026.
Peter Chen, Héloïse Briançon, Deborah Eshagh, Tali‐Anne Szwebel, Benjamin Terrier, Pierre Sohier, Rudy Birsen   +6 more
wiley   +1 more source

Successful Treatment of Refractory Sweet's Syndrome With Adalimumab in a Myelodysplastic Syndrome Patient

eJHaem, Volume 7, Issue 1, February 2026.
ABSTRACT We report a 69‐year‐old man diagnosed as myelodysplastic syndrome and administrated with azacitidine and selinexor treatment. Then he was revealed high fever and multiple tender erythematous papules involving his mouth, back, upper and lower extremities, which were diagnosed as Sweet's syndrome by skin biopsy.
Liya Ma, Yaojia Shen, Jie Chang, Shanshan Suo, Lu Wang, Weifang Zhu, Jianjun Qiao, Hongyan Tong   +7 more
wiley   +1 more source

A clinical phenotype of VEXAS syndrome with pleural effusion, infiltrates, and systemic inflammation in a 76-year-old patient: a case report

Journal of Medical Case Reports
Introduction VEXAS syndrome, characterized by a UBA1 gene mutation, is a rare and severe systemic inflammatory disease predominantly affecting men. Since its initial description in 2020, it has been noted for its broad clinical phenotype and frequent ...
Melanie Berger, Falk Schumacher, Maximilian Wollsching-Strobel, Doreen Kroppen, Sarah B. Stanzel, Daniel S. Majorski, Kathrin Fricke, Ilka Plath, Wolfram Windisch, Maximilian Zimmermann   +9 more
doaj   +1 more source

Oral mucosal manifestations with identical mutations to the bone marrow in a patient with VEXAS syndrome

Rheumatology &Autoimmunity, Volume 6, Issue 1, Page 60-62, March 2026.
Lilian Vasaitis, Lena Blomstrand, Tatjana Pandzic, Miklos Gulyas, Panagiotis Baliakas, Viktor Ljungström   +5 more
wiley   +1 more source