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Autopsy case of sudden maternal death from thrombotic thrombocytopenic purpura
Author(s) -
Yamamoto Takuma,
Fujimura Yoshihiro,
Emoto Yuko,
Kuriu Yukiko,
Iino Morio,
Matoba Ryoji
Publication year - 2013
Publication title -
journal of obstetrics and gynaecology research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 50
eISSN - 1447-0756
pISSN - 1341-8076
DOI - 10.1111/j.1447-0756.2012.01941.x
Subject(s) - medicine , adamts13 , thrombotic thrombocytopenic purpura , autopsy , von willebrand factor , thrombocytopenic purpura , cause of death , pathology , sudden death , thrombotic microangiopathy , disease , platelet
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.