In our case, the rapid clearance of anti-PLA2R Ab following acalabrutinib treatment, despite only partial control of CLL, favors the hypothesis that acalabrutinib had a direct role in controlling the immune dysregulation, a frequent feature of CLL, leading to the production of pathogenic autoantibodies and might be an interesting alternative strategy in antibodies-mediated kidney diseases like pMN. This evidence concerns the gene PLA2R1 and B-cell chronic lymphocytic leukemia.