title: Autopsy case of sudden maternal death from thrombotic thrombocytopenic purpura.
abstract: A 31-year-old pregnant woman was transferred to the emergency room at 27 weeks of gestation. She had one-day history of fever and upper abdominal pain. Soon after admission, she underwent cardiopulmonary arrest. Autopsy was performed and multiple microthrombi were seen within the small-caliber vessels of many organs, but not in the lungs. Immunohistochemical staining revealed that the thrombi were rich in von Willebrand factor. We also obtained results which showed severely deficient plasma a disintegrin-like and metalloprotease with thrombospondin motifs (ADAMTS) 13 activity and positive ADAMTS13 inhibitor, confirming a diagnosis of thrombotic thrombocytopenic purpura. As far as we know, in Japan, this is the first autopsy report of sudden maternal death from thrombotic thrombocytopenic purpura. We expect that the routine laboratory application of ADAMTS13 assays for unknown thrombocytopenic patients during pregnancy may help in differential diagnosis at an earlier stage of the disease and facilitate tailor-made therapeutic intervention.
authors: Department of Legal Medicine, Osaka University Graduate School of Medicine, Osaka Department of Blood Transfusion Medicine, Nara Medical University, Nara, Japan. yamamoto@legal.med.osaka-u.ac.jp
authors: Takuma
authors: T
authors: Yamamoto
authors: Yoshihiro
authors: Y
authors: Fujimura
authors: Yuko
authors: Y
authors: Emoto
authors: Yukiko
authors: Y
authors: Kuriu
authors: Morio
authors: M
authors: Iino
authors: Ryoji
authors: R
authors: Matoba
mesh_terms: D000328:Adult; D003645:Death, Sudden / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007564:Japan; D063130:Maternal Death; D011247:Pregnancy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000473:pathology*