A “Leopard Man” Aspect on 18F-FDG PET/CT Revealing a VEXAS Syndrome DOI

Alexandre Fagart,

T. Quéméneur,

Guillaume Collet

et al.

Clinical Nuclear Medicine, Journal Year: 2022, Volume and Issue: 48(1), P. e33 - e34

Published: Oct. 17, 2022

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an adult-onset autoinflammatory disease caused by somatic UBA1 mutations first described in 2020. Most of these patients met clinical criteria for inflammatory (relapsing polychondritis, Sweet syndrome, polyarteritis nodosa, or giant-cell arteritis) a hematologic condition (myelodysplastic multiple myeloma) both. We here FDG PET/CT "leopard man" appearance, with abnormal marrow recruitment the findings, 70-year-old man diagnosed syndrome.

Language: Английский

The First Korean Case of VEXAS Syndrome Caused by a UBA1 Somatic Variant DOI Open Access
Jihoon G. Yoon, Seungbok Lee, Sheehyun Kim

et al.

Annals of Laboratory Medicine, Journal Year: 2022, Volume and Issue: 43(2), P. 217 - 220

Published: Oct. 25, 2022

Jihoon G. Yoon, M.D., Ph.D., Seungbok Lee, Sheehyun Kim, Man Jin Yoon Hwan Chang, Kyun Park, Dong-Yeop Shin, and Jangsup Moon, Ph.D.. Ann Lab Med 2023;43:217-20. https://doi.org/10.3343/alm.2023.43.2.217

Language: Английский

Citations

7
Successful treatment with fludarabine and cyclophosphamide in a VEXAS syndrome patient with associated myelodysplastic syndrome: a case report and systematic review DOI Creative Commons

Polina Bellman,

Jesus D. Gonzalez‐Lugo, Moazzam Shahzad

et al.

Frontiers in Oncology, Journal Year: 2024, Volume and Issue: 14

Published: April 11, 2024

Vacuoles, E1 syndrome, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a chronic inflammatory disorder that affects various organ systems. It associated with hematologic malignancies and generally refractory to therapies. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) may be considered for selected patients. We report case wherein systemic hematological manifestations completely resolved in patient VEXAS myelodysplastic (MDS), following the administration of fludarabine cyclophosphamide as part preparation allo-HSCT. conducted systematic literature review included 86 patients MDS. Most cases presented musculoskeletal involvement (71%) anemia (72%) lower-risk responded corticosteroids (CS) but had recurrence symptoms CS taper were other immunosuppressive agents. Hypomethylating agents Janus kinase inhibitors achieved complete response some cases. Further research needed develop more effective treatment strategies.

Language: Английский

Citations

1
Navigating therapeutic challenges in VEXAS syndrome: exploring IL-6 and JAK inhibitors at the forefront DOI Creative Commons
Xiao Li,

Wen Hui Huang,

Xiao Yang

et al.

Molecular Medicine, Journal Year: 2024, Volume and Issue: 30(1)

Published: Sept. 17, 2024

Language: Английский

Citations

1
Somatic mutations in VEXAS Syndrome and Erdheim-Chester disease: Inflammatory myeloid diseases DOI

Pedro E. Alcedo,

Fernanda Gutierrez‐Rodrigues, Bhavisha A. Patel

et al.

Seminars in Hematology, Journal Year: 2022, Volume and Issue: 59(3), P. 156 - 166

Published: July 1, 2022

Language: Английский

Citations

5
A “Leopard Man” Aspect on 18F-FDG PET/CT Revealing a VEXAS Syndrome DOI

Alexandre Fagart,

T. Quéméneur,

Guillaume Collet

et al.

Clinical Nuclear Medicine, Journal Year: 2022, Volume and Issue: 48(1), P. e33 - e34

Published: Oct. 17, 2022

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an adult-onset autoinflammatory disease caused by somatic UBA1 mutations first described in 2020. Most of these patients met clinical criteria for inflammatory (relapsing polychondritis, Sweet syndrome, polyarteritis nodosa, or giant-cell arteritis) a hematologic condition (myelodysplastic multiple myeloma) both. We here FDG PET/CT "leopard man" appearance, with abnormal marrow recruitment the findings, 70-year-old man diagnosed syndrome.

Language: Английский

Citations

5