Myelodysplastic syndrome and autoimmune disorders: two sides of the same coin?

MJ Hochman, AE DeZern - The Lancet Haematology, 2022 - thelancet.com
Systemic inflammatory and autoimmune diseases and myelodysplastic syndromes have
been linked in individual patients and in larger case series for at least 25 years. These …

Somatic mutations in rheumatological diseases: VEXAS syndrome and beyond

KA Sikora, KV Wells, EC Bolek, AI Jones… - …, 2022 - academic.oup.com
Discovery of the VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic)
syndrome demonstrates that somatic mutations in haematological precursor cells can cause …

Early activation of inflammatory pathways in UBA1-mutated hematopoietic stem and progenitor cells in VEXAS

Z Wu, S Gao, Q Gao, BA Patel, EM Groarke, X Feng… - Cell Reports …, 2023 - cell.com
Summary VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a
pleiotropic, severe autoinflammatory disease caused by somatic mutations in the ubiquitin …

The use of immunosuppressive therapy in MDS: clinical outcomes and their predictors in a large international patient cohort

M Stahl, M DeVeaux, T De Witte, J Neukirchen… - Blood …, 2018 - ashpublications.org
Most studies of immunosuppressive therapy (IST) in myelodysplastic syndromes (MDS) are
limited by small numbers and their single-center nature, and report conflicting data …

VEXAS syndrome in myelodysplastic syndrome with autoimmune disorder

H Huang, W Zhang, W Cai, J Liu, H Wang, T Qin… - … Hematology & Oncology, 2021 - Springer
Abstract VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a
newly-described adult-onset inflammatory syndrome characterized by vacuoles in myeloid …

Molecular and clinical presentation of UBA1-mutated myelodysplastic syndromes

M Sirenko, E Bernard, M Creignou, D Domenico… - Blood, 2024 - ashpublications.org
Mutations in UBA1, which are disease-defining for VEXAS (vacuoles, E1 enzyme, X-linked,
autoinflammatory, somatic) syndrome, have been reported in patients diagnosed with …

Impact of preexisting autoimmune disease on myelodysplastic syndromes outcomes: a population analysis

D Adrianzen-Herrera, AD Sparks, R Singh… - Blood …, 2023 - ashpublications.org
Preexisting autoimmune disease affects between 10% and 30% of patients with
myelodysplastic syndromes (MDS). Studies comparing outcomes in patients with MDS with …

Somatic mutations and the risk of undifferentiated autoinflammatory disease in MDS: an under-recognized but prognostically important complication

A Watad, M Kacar, NL Bragazzi, Q Zhou… - Frontiers in …, 2021 - frontiersin.org
Objectives: We theorized that myelodysplastic syndrome (MDS) with somatic mutations and
karyotype abnormalities are associated with autoinflammation, and that the presence of …

Use of immunosuppressive therapy for management of myelodysplastic syndromes: a systematic review and meta-analysis

M Stahl, JP Bewersdorf, S Giri, R Wang… - …, 2019 - pmc.ncbi.nlm.nih.gov
Immunosuppressive therapy (IST) is one therapy option for treatment of patients with lower-
risk myelodysplastic syndromes (MDS). However, the use of several different …

Gastrointestinal Behcet's-like disease with myelodysplastic neoplasms with trisomy 8: a French case series and literature review

N Wesner, L Drevon, A Guedon, JB Fraison… - Leukemia & …, 2019 - Taylor & Francis
We report the 11 cases of+ 8-MDS/MPN associated with Behcet's-like syndrome and
compare them with Behcet's disease and Crohn's disease, pool with literature cases for …