Abstract
Hereditary thrombotic thrombocytopenic purpura (hTTP) results from mutations leading to ADAMTS13 deficiency, accumulation of ultra-large von Willebrand factor (vWF) multimers, serious thrombotic complications, and a substantial risk of severe obstetric morbidity (SOM). Patients are treated by fresh frozen plasma (FFP) transfusions during pregnancy, with variable efficacy. The objectives of our study were to determine in women with hTTP: 1) the association between SOM and non-pregnant vWF antigen levels; and 2) whether the latter can indicate the response to FFP transfusion.
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