What is it about?
This is an update of a previously reported case of thrombotic thrombocytopenic purpura (TTP) that was refractory to plasma exchange, steroids, vincristine, rituximab and N-acetylcysteine, but achieved remission after the institution of eculizumab. This case report suggested that eculizumab might be an effective treatment of TTP. However, upon further investigation, it was discovered that the patient had concurrent atypical hemolytic uremic syndrome (AHUS) due to autoantibodies of complement factor H. His persistent thrombocytopenia was due to AHUS rather than TTP. The patient achieved remission because eculizumab controlled his AHUS.
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Why is it important?
In a patient with TTP that is "refractory" to treatment, it is important to consider the existence of other causes of thrombocytopenia, hemolysis, or organ dysfunction.
Perspectives
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This page is a summary of: Use of eculizumab in the treatment of a case of refractory, ADAMTS13-deficient thrombotic thrombocytopenic purpura: additional data and clinical follow-up, British Journal of Haematology, May 2013, Wiley,
DOI: 10.1111/bjh.12387.
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