Year: 2026
Type: article
Study design: Case Report
Abstract: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) results from severe ADAMTS13 deficiency and may relapse despite modern therapy, with survival exceeding 95% in contemporary cohorts. We report the case of a 43-year-old patient with multiple clinical and ADAMTS13 relapses over 15 years, who developed intolerance or had incomplete responses to several immunosuppressive agents, including anti-CD20 and anti-CD38 therapies. Splenectomy was considered high risk due to significant cardiac comorbidity. Off-label zanubrutinib, a second-generation Bruton tyrosine kinase (BTK) inhibitor licensed locally for chronic lymphocytic leukemia (CLL) was initiated after the sixth relapse, which was a clinical one, simultaneously with plasma exchange (PEX) and corticosteroids. Following initiation, ADAMTS13 activity rose, platelet counts normalized, and the patient has remained in clinical remission for 20 months under zanubrutinib. This case illustrates the feasibility of BTK inhibition in selected, treatment-limited iTTP.
Source: Frontiers in Immunology
Language: English
Subfield: Immunology
Field: Immunology and Microbiology
Domain: Life Sciences
Open Access status: gold
Estimated APC paid: $3,801
OpenAlex ID: W7221806106