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Peer-reviewedCC BY 4.0Frontiers in Immunology

Case Report: Zanubrutinib for refractory immune thrombotic thrombocytopenic purpura

  • Adrián Dante De Angelis1,2
  • Annatina Schnegg-Kaufmann1,3
  • Elisavet Moutzouri1,4
  • Katarzyna Aleksandra Jalowiec1
  • Milos Ratknic1,5
  • Johanna A. Kremer Hovinga1,3
  • Ioannis Chanias1,6
6 affiliations
  1. 1Department of Hematology and Central Hematological Laboratory, Inselspital, Bern University Hospital, University of Bern
  2. 2Centre for Oncology and Hematology, Thun-Spiez-Berner Oberland, Spital STS AG
  3. 3Department for BioMedical Research, University of Bern
  4. 4Department of Hematology, HFR Hôpital Fribourgeois
  5. 5Centre of Oncology/Haematology and Transfusion Medicine, Cantonal Hospital Aarau
  6. 6Department of Hematology, Bone Marrow Transplantation Unit and Institute of Cell Therapy, University Hospital of Patras
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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.

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Cite

BibTeXRIS
BibTeX
@article{angelis2026case,
  title = {{Case Report: Zanubrutinib for refractory immune thrombotic thrombocytopenic purpura}},
  author = {Adrián Dante De Angelis and Annatina Schnegg-Kaufmann and Elisavet Moutzouri and Katarzyna Aleksandra Jalowiec and Milos Ratknic and Johanna A. Kremer Hovinga and Ioannis Chanias},
  journal = {Frontiers in Immunology},
  year = {2026},
  month = oct,
  doi = {10.3389/fimmu.2026.1895825},
  url = {https://www.frontiersin.org/articles/10.3389/fimmu.2026.1895825/full},
  note = {CC BY 4.0},
}
RIS
TY  - JOUR
TI  - Case Report: Zanubrutinib for refractory immune thrombotic thrombocytopenic purpura
AU  - Adrián Dante De Angelis
AU  - Annatina Schnegg-Kaufmann
AU  - Elisavet Moutzouri
AU  - Katarzyna Aleksandra Jalowiec
AU  - Milos Ratknic
AU  - Johanna A. Kremer Hovinga
AU  - Ioannis Chanias
PY  - 2026
DA  - 2026/10/14
JO  - Frontiers in Immunology
DO  - 10.3389/fimmu.2026.1895825
UR  - https://www.frontiersin.org/articles/10.3389/fimmu.2026.1895825/full
AB  - 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.
SN  - 1664-3224
ER  -