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Case of maternal and fetal deaths due to severe congenital thrombotic thrombocytopenic purpura ( U pshaw– S chulman syndrome) during pregnancy
Author(s) -
Tanaka Hirokazu,
Tenkumo Chiaki,
Mori Nobuhiro,
Kokame Koichi,
Fujimura Yoshihiro,
Hata Toshiyuki
Publication year - 2014
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/jog.12125
Subject(s) - medicine , adamts13 , thrombotic thrombocytopenic purpura , pregnancy , fulminant , gestation , antiphospholipid syndrome , fetus , thrombocytopenic purpura , obstetrics , pediatrics , platelet , thrombosis , biology , genetics
Upshaw– S chulman syndrome ( USS ) involves a congenital deficiency of von W illebrand factor‐cleaving metalloprotease ( ADAMTS13 ) activity due to gene mutations. Female patients develop overt thrombotic thrombocytopenic purpura ( TTP ) caused by a decline of ADAMTS13 activity in pregnancy. A 23‐year‐old nulliparous J apanese woman died due to severe, rapid progression of TTP with intrauterine fetal death at 20 weeks of gestation after its onset, even though she underwent intensive treatment which included plasma exchange. She had a history of idiopathic thrombocytopenic purpura at the age of 3 years. The patient's ADAMTS13 activity was of very low level. It should be borne in mind that there is the possibility of rapidly progressive fulminant USS during pregnancy.