ArticleAnnals of hematology2025
Concurrent inhibited erythropoiesis in a case of VEXAS syndrome.
Article in Annals of hematology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a rare disease described by Beck et al. in 2020. Here we report a case of VEXAS syndrome with progressive worsening of pancytopenia. This patient demonstrated inhibited erythropoiesis along with normal granulopoiesis and megakaryopoiesis in the bone marrow. A diagnosis of myelodysplastic syndrome (MDS) was ruled out, while the patient presented with pure red cell aplasia (PRCA), a manifestation not previously described in the context of VEXAS syndrome. Therefore, VEXAS syndrome may also present with PRCA-like erythroid hypoplasia, which may aid in the better recognition of this disease.
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