88yr old male with LGL from circulating blood and CBC: ANC 1300, Hb 10.9, plt 61k. Bone marrow report: Features consistent with: (1) VEXAS syndrome (Vacuoles, E1 enzyme, Xlinked, Autoinflammatory, Somatic) in a hypercellular marrow with erythroid and megakaryocytic dyspoiesis and no greater than 3% blasts. (2) Dysplastic hematopoiesis with 7q deletion consistent with MDN with low blasts and multilineage dysplasia (MDS-LB-MLD) (3) Low level T cell large granular lymphocytic leukemia (T-LGLL) Does he require treatment or rheumatology workup?
Blood counts look ok for now, so could continue to monitor. Would send NGS to assess what type of mutations are present as that may give more clarity on what is driving the disease. But given the patient's age and lower-risk MDS, may be reasonable to get mutation information and hold off on treatment until cytopenias/symptoms progress.
