Thrombotic Thrombocytopenic Purpura
Author(s) -
Stéphanie Clément-Guinaudeau,
JeanFrançois Deux,
Marc Michel,
Jérôme Garot
Publication year - 2008
Publication title -
circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 7.795
H-Index - 607
eISSN - 1524-4539
pISSN - 0009-7322
DOI - 10.1161/circulationaha.108.764696
Subject(s) - medicine , emergency department , university hospital , medical school , magnetic resonance imaging , cardiology , nuclear medicine , radiology , psychiatry , medical education
A 26-year-old black woman, without risk factors for coronary artery disease, was admitted in the cardiology department of our institution for acute chest pain. Three months before admission, she had an episode of abdominal pain along with fever, thrombocytopenia (platelet count 23 000/mm3), and hemolytic anemia (hemoglobin 5.1 g/dL). Thrombotic thrombocytopenic purpura was diagnosed with ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity of <5% and presence of anti-ADAMTS-13 antibody activity.1 Severe thrombocytopenia (<50 000/mm3) persisted despite intensive treatment with high-dose steroids, rituximab (anti-CD20 antibody), and vincristine. Splenectomy was then performed, and the patient underwent daily plasma exchange along with repeated intravenous-bolus cyclophosphamide. She was admitted in the cardiology department because of acute chest pain that lasted ≈20 minutes and was resistant to sublingual nitrate, with stable, diffuse, negative T waves on ECG (Figure 1A). The test for troponin I twice showed …
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