August 2026 Research Update

These studies add to a clearer picture that blood clots are a frequent part of VEXAS for many people, while testing in one thrombosis clinic did not find VEXAS as a hidden cause of unexplained clots in a small selected group. In an Italian group with confirmed VEXAS, about half had a clot at some point, and new clots still occurred after diagnosis. A notable detail was that some clot events happened while people were already taking anticoagulation, especially during inflammatory flares, showing how complicated clot risk can be in VEXAS. [1]

Researchers reviewed 1,086 people seen for thrombosis and identified 21 men with unprovoked thrombosis and at least one feature compatible with VEXAS for UBA1 exon 3 testing. None of those 21 had a UBA1 mutation. In contrast, among 87 people with molecularly confirmed VEXAS, 49% had had thrombosis, and most events were venous clots such as deep vein thrombosis. The study also reported a 24-month chance of a first clot after VEXAS diagnosis of 18.3% in those without a prior clot history. [1]

This cohort also described real-world anticoagulation patterns, with direct oral anticoagulants (DOACs) used most often, and it noted that some people had multiple clot events. The report described that many clot events occurred during active anticoagulation, and it also observed lower platelet counts among those receiving low molecular weight heparin compared with those receiving DOACs, though the study was not designed to prove one approach is safer or more effective. It also found that some tested patients had inherited clotting variants or antiphospholipid antibodies, but testing was only done in a subset. [1]

For patients and caregivers, this month strengthens the message that clotting can be a meaningful part of the VEXAS experience, and that risk may continue after the diagnosis is made. It also helps separate two ideas: VEXAS can involve clots for many people who already have VEXAS, but VEXAS mutations were not found when 21 men with unexplained clots and at least one VEXAS-like feature were tested. What remains uncertain is how best to match anticoagulation choices to different VEXAS situations, because this work was observational and clot events were reported even during anticoagulation, especially around flares. [1]

This month included an early signal that certain immune cell patterns might relate to who is more likely to develop HLH in VEXAS. Researchers reported that "adaptive" natural killer (NK) cell dominance was linked to HLH susceptibility in people with VEXAS. In plain terms, differences in one part of the immune system may help explain why a severe inflammatory complication happens in some patients and not others, but the evidence is still exploratory. [2]

The study focused on measuring and comparing immune cell features rather than testing a treatment, and it did not establish that the immune pattern causes HLH. It mainly adds a possible biological marker to watch in future research, especially if other groups can reproduce the same link. Details such as sample size and how well this marker separates higher-risk from lower-risk patients were not provided in the summary, so the practical usefulness is not yet clear. [2]

For patients, this finding may matter more in the future than right now, because it is a research-stage clue rather than a test that is ready for routine care. It helps clarify that HLH risk in VEXAS may relate to measurable immune differences, which could eventually support better risk prediction or earlier recognition if confirmed. What remains uncertain is whether this NK cell pattern is common, how stable it is over time, and whether it reliably signals HLH risk across different VEXAS populations. [2]

These case reports show how variable VEXAS can look in real life, and how that variability can delay diagnosis or complicate long-term control of inflammation. One report emphasized that VEXAS can occur even without macrocytosis (enlarged red blood cells), a feature many people associate with the condition. Another described improvement on glucocorticoids with relapse after tapering, echoing the pattern of symptoms returning when steroids are reduced. [3] [4]

In the report with diagnostic delay, an older man had repeated steroid-responsive inflammatory episodes with skin and lung involvement and anemia, but his anemia was not macrocytic, which contributed to delayed recognition. UBA1 testing on bone marrow later confirmed VEXAS (t41Val variant), but he died before results were available, underscoring how atypical presentations can be missed. The key message is that absence of a familiar lab clue does not rule out VEXAS in someone with a matching clinical picture. [3]

A separate case described an older man whose diagnosis was supported by characteristic bone marrow changes (vacuoles) and a UBA1 Met41Val mutation. He improved with glucocorticoids, but fever and anemia recurred months after stopping them. This report reinforces that steroids can calm symptoms in some people but may not provide lasting control after tapering. [4]

One case report focused on treatment with azacitidine (a drug used for certain bone marrow disorders) in a person with VEXAS who also had ETV6 and EZH2 mutations. After four courses of azacitidine given with corticosteroids, symptoms improved, the measured UBA1 mutation level fell, and the ETV6 and EZH2 mutations were no longer detected, with no symptom recurrence reported during six months of follow-up. Because it is one case, it does not show how often this response happens, but it adds to the set of real-world treatment experiences being documented. [5]

For patients and caregivers, these reports mainly add understanding rather than changing what should happen in routine care. They help clarify that VEXAS can present without macrocytosis and that symptoms can return after steroid tapering in some individuals, which may explain why diagnosis and long-term symptom control can be challenging. They also add one more example of azacitidine being used with reported benefit, but it remains uncertain which patients are most likely to respond because the evidence here is limited to a single person. [3] [4] [5]