Among the patients with UBA1 variants of unknown origin or putative germline, Graves’ disease with recurrent thyroid nodules development after goiter operation was documented for one MDS patient (L568V) and there were two additional patients with thyroid involvement (hypothyroidism, R784Q, CLL; intrathyroidal parathyroid carcinoma, N139K, MDS). Here, UBA1 is linked to B-cell chronic lymphocytic leukemia.