Systemic lupus erythematosus-associated thrombotic thrombocytopenic purpura (SLE-TTP) is a rare but potentially life-threatening condition that is characterized by thrombocytopenia, microangiopathic hemolytic anemia, and multiorgan involvement. Here, we describe a refractory case of SLE-TTP in a 38-year-old Japanese woman who was unresponsive to plasma exchange and glucocorticoid therapy. Combination treatment with caplacizumab and rituximab (RTX) leads to rapid platelet recovery, resolution of neurological symptoms, and sustained remission. Caplacizumab provided immediate inhibition of microthrombus formation, whereas RTX targeted the underlying autoimmune process through B cell depletion. Following treatment, ADAMTS13 activity normalized, allowing corticosteroid tapering and the maintenance of long-term remission. This case highlights the value of early combination therapy for refractory SLE-TTP, and suggests that early combination therapy with caplacizumab and rituximab may act synergistically to improve outcomes.
Honda et al. (Fri,) studied this question.