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Thrombotic thrombocytopenic purpura with unusual 33 recurrences: a case report

Fouad N Boctor1, Ion Chiosea, Thomas Bradley

  • 1Department of Pathology and Division of Hematology, North Shore University Hospital, Hofstra North Shore-LIJ School of Medicine, Manhasset, NY 10030, USA. fboctor@nshs.edu

Annals of Clinical and Laboratory Science
|March 7, 2013
PubMed

The hereditary or acquired deficiency of ADAMTS-13 activity leads to an excess of high molecular weight von Willebrand factor multimers in plasma, leading to platelet aggregation and diffuse intravascular thrombus formation, resulting in thrombotic thrombocytopenic purpura (TTP). We report a 36 year old male with a long history of TTP associated with 33 relapses. As a result of early transfusions, the patient acquired Hepatitis C. This time, the patient presented with a TTP relapse after a 10 year remission, following PEG-interferon-Alpha (IFA) therapy for Hepatitis C. Since IFA has been reported to activate autoimmune reactions, it may have augmented production of ADAMTS-13 antibody.

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