Stefan Zschiedrich, MD; Eric P. Prager, MD; E. Wolfgang Kuehn, MD
Potential Conflicts of Interest: Other (money to institution): Alexion Pharmaceuticals.
Zschiedrich S., Prager E., Kuehn E.; Successful Treatment of the Postpartum Atypical Hemolytic Uremic Syndrome With Eculizumab. Ann Intern Med. 2013;159:76. doi: 10.7326/0003-4819-159-1-201307020-00023
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Published: Ann Intern Med. 2013;159(1):76.
Background: Thrombocytopenia with intravascular hemolysis is a serious complication of pregnancy. When it occurs in the second and third trimesters, it is usually associated with near-absent levels of the ADAMTS13 protease that cleaves von Willebrand factor, a condition that is typical of idiopathic thrombotic thrombocytopenic purpura (1). During pregnancy, approximately 50% of cases of thrombotic thrombocytopenic purpura result in perinatal fetal loss and maternal mortality is also substantial, although plasma exchange has reduced maternal mortality from 58% before 1980 to 9% after 1996 (1). In contrast, when thrombocytopenia with intravascular hemolysis occurs in the postpartum period, ADAMTS13 activity is normal, and end-stage renal failure occurs within months in nearly all patients and is not prevented by plasma exchanges. This condition is known as the postpartum atypical hemolytic uremic syndrome (p-aHUS), and most patients have mutations in proteins of the alternative complement pathway, usually affecting complement factor H (2).
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Hematology/Oncology, Nephrology, Red Cell Disorders, Platelet Disorders.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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