While comprehensive studies on the genetic factors associated with thrombotic risk in VEXAS syndrome remain scarce, we wish to highlight the following findings: one patient tested positive for lupus anticoagulant (LAC) and anti-cardiolipin IgG at 140 GPL/mL (with negative results for IgM and anti-Beta2GP1); another patient was found to be a carrier of HLA-B51, for whom Behçet’s disease had initially been suspected; and a third patient presented with both a mutation in UBA1 and a mutation in CECR1 (or ADA2), a gene associated with DADA2 syndrome. Here, CD40LG is linked to VEXAS syndrome.