ADAMTS13 autoantibody-expressing B cell

ADAMTS13 autoantibody-expressing B cells are autoreactive B lymphocytes that produce anti-ADAMTS13 autoantibodies, primarily IgG, which inhibit ADAMTS13 enzymatic activity, induce an open ADAMTS13 conformation, and promote its clearance from circulation. These cells differentiate into antibody-producing plasma cells or memory B cells in germinal centers, driving immune-mediated thrombotic thrombocytopenic purpura (iTTP) through accumulation of ultra-large von Willebrand factor multimers, microthrombi formation, thrombocytopenia, hemolytic anemia, and organ ischemia. Autoantibodies often target multiple ADAMTS13 domains, especially spacer, cysteine-rich, and C-terminal regions, with prevalence in acute and remission phases.

Other names

anti-ADAMTS13 autoantibody-producing B cellsautoreactive B cells against ADAMTS13B lymphocytes producing anti-ADAMTS13 autoantibodies