Results 21 to 30 of about 940 (155)

Successful treatment with fludarabine and cyclophosphamide in a VEXAS syndrome patient with associated myelodysplastic syndrome: a case report and systematic review

Frontiers in Oncology
Vacuoles, E1 syndrome, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a chronic inflammatory disorder that affects various organ systems. It is associated with hematologic malignancies and is generally refractory to therapies.
Polina Bellman, Jesus D. Gonzalez-Lugo, Moazzam Shahzad, Moazzam Shahzad, Muhammad Kashif Amin, Muhammad Fareed Khalid, Nahid Suleman, Nausheen Ahmed, Anurag K. Singh, Abdulraheem Yacoub, Da Zhang, Joseph P. McGuirk, Muhammad Umair Mushtaq   +12 more
doaj   +3 more sources

A rare case of VEXAS syndrome. [PDF]

Rheumatol Adv Pract
Gupta K, El-Ghazali S, Kubba F.
europepmc   +3 more sources

VEXAS Syndrome

Blood, 2021
VEXAS syndrome is a monogenic disease of adulthood caused by somatic mutations in UBA1 in hematopoietic progenitor cells. Patients present with a range of inflammatory and hematologic symptoms (Visual Abstract). Myeloid-driven autoinflammation and progressive bone marrow failure lead to substantial morbidity and mortality. Effective medical treatments,
Grayson, Peter C., Patel, Bhavisha A., Young, Neal S.   +2 more
openaire   +2 more sources

Aortitis in VEXAS Syndrome. [PDF]

Intern Med
Tsuchiya J, Nunokawa T, Sogabe S, Chinen N.   +3 more
europepmc   +3 more sources

An update on VEXAS syndrome

Expert Review of Clinical Immunology, 2022
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently described, late-onset, acquired autoinflammatory disorder caused by mutations in the UBA1 gene. The various clinical manifestations of VEXAS broadly divided into inflammatory or haematological.
Al-Hakim, A, Savic, S
openaire   +3 more sources

Distinction between clonal and paraclonal cutaneous involvements in VEXAS syndrome

Experimental Hematology & Oncology, 2022
VEXAS (vacuoles, E1 enzyme, X-linked, auto-inflammatory, somatic) syndrome is an inflammatory disorder with hematological and systemic features. A recent study demonstrated that the dermal infiltrate in neutrophilic dermatosis from VEXAS patients is ...
Valentin Lacombe, Annaelle Beucher, Geoffrey Urbanski, Yannick Le Corre, Laurane Cottin, Anne Croué, Anne Bouvier   +6 more
doaj   +1 more source

Recurrent superior orbital fissure syndrome associated with VEXAS syndrome

Journal of Ophthalmic Inflammation and Infection, 2023
Purpose To describe a case of recurrent orbital inflammation and superior orbital fissure syndrome associated with VEXAS (vacuoles, E1 enzyme, X-linked, auto-inflammatory, somatic) syndrome. Case presentation VEXAS syndrome is a recently identified multi-
Katie Myint, Namritha Patrao, Oana Vonica, Kaveh Vahdani   +3 more
doaj   +1 more source

VEXAS-Syndrom

Zeitschrift für Rheumatologie
The VEXAS (vacuoles, E1 enzyme, X‑linked, autoinflammatory, somatic) syndrome is an acquired autoinflammatory disease, which is based on somatic mutations in the UBA1 gene and described as an hematoinflammatory disease. It is clinically expressed as an inflammatory systemic disease with general vegetative symptoms and organ manifestations of the skin ...
M, Krusche, N, Ruffer, I, Kötter, K, Sockel   +3 more
  +6 more sources

VEXAS syndrome in myelodysplastic syndrome with autoimmune disorder

Experimental Hematology & Oncology, 2021
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly-described adult-onset inflammatory syndrome characterized by vacuoles in myeloid and erythroid precursor cells and somatic mutations affecting methionine-41 (p.Met41) in
Huijun Huang, Wenjun Zhang, Wenyu Cai, Jinqin Liu, Huijun Wang, Tiejun Qin, Zefeng Xu, Bing Li, Shiqiang Qu, Lijuan Pan, Gang Huang, Robert Peter Gale, Zhijian Xiao   +12 more
doaj   +1 more source

VEXAS Syndrome: An Overlooked Threat. [PDF]

Balkan Med J
Uğurlu S, Kılıç B.
europepmc   +5 more sources