{
  "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.",
  "abstract_shown": true,
  "affiliations": [
    "Department of Hematology and Central Hematological Laboratory, Inselspital, Bern University Hospital, University of Bern",
    "Centre for Oncology and Hematology, Thun-Spiez-Berner Oberland, Spital STS AG",
    "Department for BioMedical Research, University of Bern",
    "Department of Hematology, HFR Hôpital Fribourgeois",
    "Centre of Oncology/Haematology and Transfusion Medicine, Cantonal Hospital Aarau",
    "Department of Hematology, Bone Marrow Transplantation Unit and Institute of Cell Therapy, University Hospital of Patras"
  ],
  "article_type": "case report",
  "arxiv": null,
  "author_total": 7,
  "authors": [
    {
      "affs": [
        0,
        1
      ],
      "name": "Adrián Dante De Angelis"
    },
    {
      "affs": [
        0,
        2
      ],
      "name": "Annatina Schnegg-Kaufmann"
    },
    {
      "affs": [
        0,
        3
      ],
      "name": "Elisavet Moutzouri"
    },
    {
      "affs": [
        0
      ],
      "name": "Katarzyna Aleksandra Jalowiec"
    },
    {
      "affs": [
        0,
        4
      ],
      "name": "Milos Ratknic"
    },
    {
      "affs": [
        0,
        2
      ],
      "name": "Johanna A. Kremer Hovinga"
    },
    {
      "affs": [
        0,
        5
      ],
      "name": "Ioannis Chanias"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/5mk0rp6g562v3.bib",
  "cited_by": 0,
  "doi": "10.3389/fimmu.2026.1895825",
  "fulltext_url": "https://europepmc.org/articles/PMC13713702",
  "id": "5mk0rp6g562v3",
  "issns": [
    "1664-3224"
  ],
  "landing_url": "https://www.frontiersin.org/articles/10.3389/fimmu.2026.1895825/full",
  "language": "eng",
  "licence": "CC-BY-4.0",
  "licence_label": "CC BY 4.0",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "heading",
      "Case Report"
    ],
    [
      "openalex",
      "2403|Complement system in diseases"
    ]
  ],
  "openalex": "W7221806106",
  "pdf_url": "https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2026.1895825/pdf",
  "pmcid": "PMC13713702",
  "pmid": "42952733",
  "published": "2026-10-14",
  "publisher": "Frontiers Media SA",
  "ris": "https://research.draconitic.com/paper/5mk0rp6g562v3.ris",
  "sources": [
    {
      "first_seen": "2026-10-30T12:19:17Z",
      "native_id": "10.3389/fimmu.2026.1895825",
      "source": "crossref",
      "url": "https://www.frontiersin.org/articles/10.3389/fimmu.2026.1895825/full"
    },
    {
      "first_seen": "2026-10-31T00:20:41Z",
      "native_id": "MED:42952733",
      "source": "europepmc",
      "url": "https://europepmc.org/article/MED/42952733"
    },
    {
      "first_seen": "2026-10-31T12:24:12Z",
      "native_id": "PMC13713702",
      "source": "pmc",
      "url": "https://doi.org/10.3389/fimmu.2026.1895825"
    }
  ],
  "subjects": [
    {
      "field": "biology",
      "origin": "openalex:2403|Complement system in diseases",
      "subfield": "immunology"
    }
  ],
  "title": "Case Report: Zanubrutinib for refractory immune thrombotic thrombocytopenic purpura",
  "title_text": "Case Report: Zanubrutinib for refractory immune thrombotic thrombocytopenic purpura",
  "type": "peer-reviewed",
  "updated_at": "2026-11-01T12:21:19Z",
  "url": "https://research.draconitic.com/paper/5mk0rp6g562v3",
  "venue": "Frontiers in Immunology",
  "versions": []
}