| Pregnancy-associated thrombotic thrombocytopenic purpura with anti-centromere antibody-positive Raynaud's Syndrome. | |
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MedLine Citation:
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PMID: 20558950 Owner: NLM Status: In-Process |
Abstract/OtherAbstract:
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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. |
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Authors:
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Ryu Watanabe; Tsuyoshi Shirai; Yumi Tajima; Hiroto Ohguchi; Yasushi Onishi; Hiroshi Fujii; Naruhiko Takasawa; Tomonori Ishii; Hideo Harigae |
Publication Detail:
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Type: Case Reports; Journal Article Date: 2010-06-15 |
Journal Detail:
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Title: Internal medicine (Tokyo, Japan) Volume: 49 ISSN: 1349-7235 ISO Abbreviation: Intern. Med. Publication Date: 2010 |
Date Detail:
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Created Date: 2010-06-18 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9204241 Medline TA: Intern Med Country: Japan |
Other Details:
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Languages: eng Pagination: 1229-32 Citation Subset: IM |
Affiliation:
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Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Japan. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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