title: Pregnancy-associated thrombotic thrombocytopenic purpura with anti-centromere antibody-positive Raynaud's syndrome.
abstract: Thrombotic thrombocytopenic purpura (TTP), scleroderma renal crisis (SRC), and hemolysis, elevated liver enzyme levels, and a low platelet count (HELLP) syndrome display common symptoms that include microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. Therefore, it is important to distinguish between them because their treatments vary: however, the differential diagnosis is sometimes difficult. We report a 32-year-old woman who was referred to our department for further examination of microangiopathic hemolytic anemia, thrombocytopenia, and a slightly elevated serum creatinine level with anti-centromere antibody-positive Raynaud's syndrome in the early puerperal period. TTP, SRC, and HELLP syndrome were considered in the differential diagnosis, but the measurement of a disintegrin-like metalloprotease with thrombospondin type 1 motifs 13 (ADAMTS 13) activity and its inhibitor level led to the diagnosis of TTP. She was successfully treated by plasma exchange and high-dose prednisolone and angiotensin-converting enzyme inhibitor. If microangiopathic hemolytic anemia and thrombocytopenia are observed in perinatal women or patients with signs of systemic sclerosis, the measurement of ADAMTS13 activity and its inhibitor level are essential for diagnosis and therapeutic choice.
authors.affiliation: Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Ryu
authors.initials: R
authors.lastname: Watanabe
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Shirai
authors.forename: Yumi
authors.initials: Y
authors.lastname: Tajima
authors.forename: Hiroto
authors.initials: H
authors.lastname: Ohguchi
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Onishi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Fujii
authors.forename: Naruhiko
authors.initials: N
authors.lastname: Takasawa
authors.forename: Tomonori
authors.initials: T
authors.lastname: Ishii
authors.forename: Hideo
authors.initials: H
authors.lastname: Harigae
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000096:biosynthesis* / Q000097:blood; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000097:blood* / Q000175:diagnosis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood* / Q000150:complications / Q000175:diagnosis; D011928:Raynaud Disease / Q000097:blood* / Q000150:complications / Q000175:diagnosis
chemical_list: D000974:Antibodies, Antinuclear; C538950:anticentromere antibody