Teclistamab for the treatment of autoantibody-mediated coagulation disorders

Teclistamab is a bispecific monoclonal antibody that targets B cell maturation antigen (BCMA) on plasma cells and other B lineage cells and CD3 on T cells, thereby redirecting cytotoxic T cells to eliminate BCMA-expressing antibody-producing cells. Autoantibody-mediated coagulation disorders are often driven by long-lived antibody-producing cells that may persist despite conventional immunosuppression or B cell depletion.

To investigate whether teclistamab represents a treatment option for patients with refractory or rapidly progressive autoantibody-mediated coagulation disorders.

We retrospectively analyzed seven patients with autoantibody-mediated coagulation disorders treated with teclistamab. Diagnoses included acquired von Willebrand disease (n=2), acquired factor XI deficiency (n=2), and catastrophic antiphospholipid syndrome (CAPS; n=3). Five patients had an underlying paraprotein, whereas two CAPS patients had no detectable paraprotein.

All seven patients achieved complete remissions (median time to remission: 24 weeks [range: 2-35]) following teclistamab. In the four patients with acquired von Willebrand disease or factor XI deficiency, improvement in the disease-defining coagulation parameter and cessation of bleeding were observed following initiation of teclistamab. In the three patients with CAPS disappearance of antiphospholipid antibodies or normalization of the aPTT-LA was observed and accompanied by clinical stabilization without further thromboembolic events. Treatment was generally well tolerated. Three patients developed grade 1 cytokine release syndrome. One patient developed transient colitis while another patient experienced reversible seronegative inflammatory joint symptoms. All patients received immunoglobulin replacement.

Teclistamab treatment was associated with complete remissions in patients with autoantibody-mediated coagulation disorders. These observations warrant further evaluation of teclistamab in selected patients.