Pregnancy-associated thrombotic thrombocytopenic purpura with anti-centromere antibody-positive Raynaud's syndrome

Ryu Watanabe, Tsuyoshi Shirai, Yumi Tajima, Hiroto Ohguchi, Yasushi Onishi, Hiroshi Fujii, Naruhiko Takasawa, Tomonori Ishii, Hideo Harigae

Research output: Contribution to journalArticlepeer-review

6 Citations (Scopus)

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 bemicroangiopathic tween them because their treatments vary: however, the differential diagnosis is sometimes difficult. We remicroangiopathic port 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 thrombospondintype 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.

Original languageEnglish
Pages (from-to)1229-1232
Number of pages4
JournalInternal Medicine
Volume49
Issue number12
DOIs
Publication statusPublished - 2010

Keywords

  • Anti-centromere antibody (ACA)
  • Pregnancy
  • Thrombotic thrombocytopenic purpura (TTP)

ASJC Scopus subject areas

  • Internal Medicine

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