Ash Lee Manley

4. Materials and Methods 4.1. Animals and Exosome Preparation Inbred B6 and congenic C.B10 mice originally obtained from the Jackson Laboratory (Bar Harbor, ME, USA) were bred and housed at a specific-pathogen-free National Institutes of Health animal facility with standard care and nutrition. Mice were used at 2–5 months of age and all animal studies were preapproved by the Animal Care and Use Committee at the National Heart, Lung, and Blood Institute.

Highlights • Inflammation and myeloid dominance originate in hematopoietic stem cells in VEXAS syndrome • mtUBA1 myeloid cells upregulate inflammatory pathways compared with wild-type cells • There are biased granulocytic differentiation and increased proliferation of mtUBA1 cells • Apoptosis of mtUBA1 lymphoid progenitors causes impaired lymphocytopoiesis in disease Summary VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a pleiotropic, severe autoinflammatory...

RESEARCH ARTICLE This study was presented as a poster at the European Hematology Association 2022 Congress. Abstract Immune severe aplastic anemia (SAA) is characterized by pancytopenia and immune-mediated bone marrow destruction. SAA may be treated with hematopoietic stem cell transplantation (HSCT) or immunosuppressive therapy (IST). However, 30% of patients treated with IST relapse.