Hereditary thrombotic thrombocytopenic purpura acquired through liver transplantation: A case report

Karlyn A. Martin*, Anaadriana Zakarija, Oluwatobi Odetola, Dinee Simpson, Amanda Cheung, Erin Kinsella, Satish Nadig, Juan Caicedo, Regina Stein

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


A 62-year-old received orthotopic liver transplantation. Three weeks later, thrombotic microangiopathy developed. Testing revealed thrombotic thrombocytopenic purpura (TTP) characterized by low ADAMTS13 (A Disintegrin-like Metallopeptidase with ThromboSpondin type 1 motif 13) activity and no inhibitor of ADAMTS13 protein. Retrospective attainment of donor records revealed a TTP diagnosis, presumably hereditary TTP (hTTP), as an ADAMTS13 protein inhibitor was not mentioned. As the grafted liver does not produce ADAMTS13 protein, the recipient now functionally has hTTP and will likely need plasma transfusions indefinitely. While hTTP is extremely rare, it should be considered a contraindication to liver donation outside of exceptional circumstances. If a potential liver donor has TTP listed on medical history, attempts should be made to determine whether it is autoimmune or hereditary. An accurate medical history is critical as it is the only reliable way to identify hTTP, as outside of acute exacerbations of TTP, donors with hTTP can have normal laboratory values, including normal hemoglobin, platelets, and renal function.

Original languageEnglish (US)
Pages (from-to)437-439
Number of pages3
JournalAmerican Journal of Transplantation
Issue number3
StatePublished - Mar 2023


  • coagulation and hemostasis
  • complication
  • donors and donation: donor evaluation
  • liver allograft function/dysfunction
  • organ acceptance

ASJC Scopus subject areas

  • Transplantation
  • Pharmacology (medical)
  • Immunology and Allergy


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