ArticleExperimental hematology & oncology2021
VEXAS syndrome in myelodysplastic syndrome with autoimmune disorder.
Article in Experimental hematology & oncology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 1 of them a synthesis that pooled it.
What it found
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
25 citing papers in PubMed, 1 synthesis or guideline pooled it, 59 citations in OpenAlex.
- Contemporary Approach to the Diagnosis and Classification of Myelodysplastic Neoplasms/Syndromes-Recommendations From the International Consortium for Myelodysplastic Neoplasms/Syndromes (MDS [icMDS]).Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2024Guideline
- What Explains the Pattern of Fibrosis in Chronic Graft-Versus-Host Disease?Clinical transplantation · 2026Review
- The profile of clinical and laboratory features of Chinese VEXAS syndrome patients with hematological abnormalities: a single-center case series.Frontiers in immunology · 2026Article
- The First Reported Case of VEXAS Syndrome in Lebanon: Efficacy of Azacitidine as a Therapeutic Option-Case Report.Case reports in hematology · 2026Article
- VEXAS syndrome caused by aGenes & diseases · 2025Article
- Refractory fever, pulmonary infiltrates, and auricular chondritis: a rare case of VEXAS syndrome following COVID-19 infection in the emergency department.World journal of emergency medicine · 2025Article
- Ocular and orbital manifestations in VEXAS syndrome.Eye (London, England) · 2024Article
- Autoimmunity and Autoinflammation: Relapsing Polychondritis and VEXAS Syndrome Challenge.International journal of molecular sciences · 2024Review
- VEXAS and Myelodysplastic Syndrome: An Interdisciplinary Challenge.Journal of clinical medicine · 2024Article
- Review
- Targeting enhanced cell death represents a potential therapeutic strategy for VEXAS syndrome.Rheumatology advances in practice · 2024Article
- Case report: VEXAS as an example of autoinflammatory syndrome in pulmonology clinical practice.Frontiers in medicine · 2024Article
- Successful treatment with fludarabine and cyclophosphamide in a VEXAS syndrome patient with associated myelodysplastic syndrome: a case report and systematic review.Frontiers in oncology · 2024Article
- Use of flow cytometric light scattering to recognize the characteristic vacuolated marrow cells in VEXAS syndrome.Blood advances · 2023Article
- Severe inflammatory disorder in trisomy 8 without myelodysplastic syndrome and response to methylprednisolone: A case report.World journal of clinical cases · 2023Article
- VEXAS Syndrome-Review.Global medical genetics · 2023Review
- VEXAS syndrome: Current clinical, diagnostic and treatment approaches.Intractable & rare diseases research · 2023Review
- Successful azacitidine therapy for myelodysplastic syndrome associated with VEXAS syndrome.International journal of hematology · 2023Article
- Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis.Rheumatology (Oxford, England) · 2023Review
- VEXAS Syndrome in a Patient with Myeloproliferative Neoplasia.Case reports in hematology · 2023Article
Corrections and comments
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Authors and funding
13 authors at 3 institutions in 3 countries.
Funding
Abstract
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 UBA1. The VEXAS syndrome often overlaps with myelodysplastic syndromes (MDS) with autoimmune disorders (AD). By screening the UBA1 gene sequences derived from MDS patients with AD from our center, we identified one patient with a p.Met41Leu missense mutation in UBA1, who should have been diagnosed as MDS comorbid with VEXAS syndrome. This patient respond poorly to immune suppressive drugs. Patients with MDS and AD who have characteristic vacuoles in myeloid and erythroid precursor cells should be screened for UBA1 mutation, these patients are likely to have VEXAS syndrome and unlikely to improve with immunosuppressive drugs and should be considered for other alternative therapies.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.