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International Angiology 2009 August;28(4):336-9

Copyright © 2009 EDIZIONI MINERVA MEDICA

language: English

Minor stroke as singular manifestation of hereditary thrombotic thrombocytopenic purpura in a young man

Lindblom A. 1, Thorsen S. 2, Hillarp A. 3, Björk P. 4

1 Section of Haematology and Coagulation Disorders, Department of Medicine, Malmö University Hospital, Malmö, Sweden 2 Hemostasis and thrombosis Lab, Department of Clinical Biochemistry, Diagnostic Centre, Rigshospitalet, Copenhagen, Denmark 3 Coagulation Laboratory, Malmö University Hospital, Malmö, Sweden 4 Hemoimmunotherapy Unit, Department of Nephrology and Transplantation, Malmö University Hospital, Malmö, Sweden


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The authors describe a case of a 38-year-old male with minor stroke due to exacerbation of hereditary deficiency of ADAMTS 13 resulting in a chronic relapsing form of thrombotic thrombocytopenic purpura (TTP). The clue to the unusual pathogenesis was given by laboratory findings of a mild anaemia and thrombocytopenia. After two days of observation, the patient was treated with plasmapheresis resulting in normalized platelet levels and continued clinical improvement. Subsequent clinical and laboratory investigation verified the diagnosis and the patient was put on regular treatments with plasma substitution.

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