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• W2085267997 abstract "It is widely acknowledged that severely persistent postpartum HELLP (Haemolysis, Elevated Liver enzymes, Low Platelet count) Syndrome (HS) shares links with the thrombotic microangiopathies, namely with Thrombotic Thrombocytopenic Purpura (TTP) which is one of its great “imitators” (Schwartz & Brenner, 1978; Martin & Stedman, 1991; Sibai, 2007). Therefore, the use of plasma exchange has been proposed for this condition, as is recommended in cases of TTP (George, 2010), but no randomised controlled trial data are available (Martin et al, 1990). Moreover, ADAM metallopeptidase with thrombospondin type 1 motif, 13 (ADAMTS13) activity has been found at low levels in HS, but not to the same extent as in TTP syndrome (Lattuada et al, 2003) and, to date, no results have been reported from samples taken in the postpartum period. We report the results of ADAMTS13 activity in five patients with persistently severe postpartum HS, all of whom had a platelet count below 50 × 109/l (Table 1). All patients received high dose steroids, as recommended by Martin et al (2006). ADAMTS13 activity was measured between days one and nine after delivery and was found at a low level in every case. Anti-ADAMTS13 plasma inhibitor was also sought when severe ADAMTS13 deficiency (below 10% of normal activity) was found. ADAMTS13 activity was undetectable in Case 5, leading to a diagnosis of TTP that was severe enough to immediately initiate daily plasma exchanges, resulting in a full remission. No ADAMTS13 plasma inhibitor was found in this case with severe ADAMTS13 deficit. On the other hand, in the four other cases, ADAMTS13 activity was diminished but detectable, between 20 and 50% (normal range, 50–150%), excluding a diagnosis of TTP. Therefore, these patients were diagnosed as “pure” cases of persistently severe postpartum HS and eventually were cured without any plasma exchanges. Platelet count returned above 100 × 109/l in all those four patients within seven days. As suggested by our results and previously published data (Lattuada et al, 2003), ADAMTS13 activity level is really low in severe HS, but not low enough to suggest TTP. Thus, we believe that, in every day clinical practice, ADAMTS13 activity may help in decisions regarding therapy when it appears very difficult, and sometimes even impossible, to distinguish HS from TTP (Rehberg et al, 2006; Keiser et al, 2012). Indeed, undetectable ADAMTS13 activity may lead to the initiation (or continuation) of plasma exchange, whereas low but detectable ADAMTS13 activity may point in favour of HS rather than TTP, indicating that plasma exchange should not be continued. To conclude, we would recommend that ADAMTS13 activity should be assessed in all patients with persistently severe postpartum HS. OP recruited the patients in the Intensive Care Unit and wrote the draft; RC reviewed the draft of the manuscript and table; FP recruited the patients in the High-risk Pregnancy Unit and reviewed the draft of the manuscript. None." @default.
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• W2085267997 date "2013-07-24" @default.
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• W2085267997 title "ADAMTS13 deficiency in severe postpartum HELLP syndrome" @default.
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• W2085267997 doi "https://doi.org/10.1111/bjh.12494" @default.
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