B-cell receptor specific for ADAMTS13 (ADAMTS13-specific BCR)

The B-cell receptor specific for ADAMTS13 is a membrane-bound immunoglobulin expressed on the surface of B lymphocytes that selectively recognizes and binds to the ADAMTS13 metalloprotease (PMID: 31570648). In patients with immune-mediated thrombotic thrombocytopenic purpura (iTTP), these specific B cells are activated and differentiate into plasma cells that secrete inhibitory autoantibodies against ADAMTS13 (PMID: 28232465). This leads to a severe deficiency of the enzyme, preventing the cleavage of ultra-large von Willebrand factor (ULVWF) multimers and resulting in systemic microvascular thrombosis and hemolytic anemia (PMID: 32810224). Current therapeutic strategies primarily utilize broad B-cell depletion with anti-CD20 monoclonal antibodies like rituximab to reduce the autoantibody burden (PMID: 27034449). Emerging precision therapies, such as Chimeric Autoantibody Receptor (CAAR) T-cell therapy, are being investigated to specifically target and eliminate only the B cells expressing these ADAMTS13-specific receptors, potentially offering a curative approach without the risks of global immunosuppression (PMID: 31243132). Monitoring ADAMTS13 activity and autoantibody titers remains the gold standard for assessing treatment efficacy and predicting disease relapse (PMID: 24935957).

Other names

Anti-ADAMTS13 B-cell receptorADAMTS13-reactive B-cell receptorADAMTS13-specific BCR

Depletion of B-cell populations or selective elimination of autoreactive B-cell clones to suppress the production of anti-ADAMTS13 autoantibodies.