Pregnancy-associated thrombotic thrombocytopenic purpura with anti-centromere antibody-positive Raynaud's syndrome.
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.
Watanabe
Ryu
R
Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Shirai
Tsuyoshi
T
Tajima
Yumi
Y
Ohguchi
Hiroto
H
Onishi
Yasushi
Y
Fujii
Hiroshi
H
Takasawa
Naruhiko
N
Ishii
Tomonori
T
Harigae
Hideo
H
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
D000974:Antibodies, Antinuclear; C538950:anticentromere antibody