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Upshaw-Schulman syndrome is an inherited form of thrombotic thrombocytopenic purpura (TTP) resulting from mutations in the ADAMTS13 gene. It is uncommon compared with acquired TTP, which often results from the presence of autoantibodies to ADAMTS13. TTP that occurs during pregnancy is usually considered acquired TTP. But can pregnancy also unmask the hereditary form of the disease?
To examine this possibility, investigators in France studied 280 women (age, <45) with severe ADAMTS13 deficiency (activity <10%) who received a diagnosis of TTP during a 10-year period.
Results were as follows:
42 women experienced their first manifestations of TTP during pregnancy or the postpartum period.
23 of those 42 women had autoantibodies to ADAMTS13, but th…