A case of severe ADAMTS 13 deficiency presenting as thrombotic thrombocytopenic purpura in pregnancy
Author(s) -
Marinos Nikolaou,
Μαρίνα Καρακάντζα,
George Adonakis,
Γεώργιος Θεοδώρου,
Nikolaos Zoumbos,
George Decavalas
Publication year - 2012
Publication title -
medicinski pregled
Language(s) - English
Resource type - Journals
eISSN - 1820-7383
pISSN - 0025-8105
DOI - 10.2298/mpns1210436n
Subject(s) - medicine , adamts13 , thrombotic thrombocytopenic purpura , pregnancy , microangiopathic hemolytic anemia , von willebrand factor , gestation , thrombocytopenic purpura , pediatrics , platelet , biology , genetics
Thrombotic thrombocytopenic purpura is a rare life-threatening disorder characterized by thrombocytopenia and microangiopathic hemolytic anemia. It is caused by the absent or severe deficiency of the von Willebrand Factor-cleaving protease named ADAMTS13. Pregnancy is a well recognized factor precipitating the appearance of the disease both in women that had reduced levels of ADAMTS13 activity prior to gestation and in those with other inherited or acquired thrombophilic syndromes.
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