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• W1969804753 abstract "Thrombotic thrombocytopenic purpura (TTP) is a rare disease characterized by thrombocytopenia and microangiopathic hemolytic anemia. Acute TTP manifests itself by fever, neurological dysfunction and renal insufficiency. It can present either as primary TTP, TTP without any demonstrable causes, or as a secondary effect of underlying diseases like sepsis, malignant hypertension, cancer, eclampsia, bone marrow transplantation, and the hemolytic–uremic syndrome (HUS) [1-3]. Plasma exchange reduced the mortality rate of primary TTP from 90% to approximately 20% [4], but survival is occasionally associated with severe complications [5]. Furthermore, plasma exchange is not always effective in other diseases bearing TTP symptoms [2, 3]. As the diagnosis ‘thrombocytopenia and hemolytic anemia’ does not distinguish TTP from other diseases, additional criteria are necessary to be able to discriminate between both in order to start a tailor-made treatment rapidly. The efficacy of plasma exchange was elucidated by the discovery that TTP patients are deficient in the von Willebrand factor (VWF) cleaving protease [6] that was later found to be a member of the metalloprotease family named ADAMTS-13 [7]. Plasma exchange is thought to supply patients with ADAMTS-13 and/or remove neutralizing autoantibodies against ADAMTS-13. ADAMTS-13 regulates the size of VWF and its deficiency results in unusually large multimers of VWF (ULVWF). These ULVWF can induce thrombosis by platelet agglutination. It was suggested that severe ADAMTS-13 deficiency (<5–10%) is a good additional criterion for TTP, although the specificity and sensitivity of ADAMTS-13 deficiency for TTP remains controversial [8-10]. Several assays have been developed for detection of ADAMTS-13 in plasma, among which the collagen-binding assay [11], the ristocetin-cofactor assay [12] and the proteolytic multimer assay [2]. In the latter, patient plasma is incubated with VWF and VWF multimers are separated by SDS-agarose followed by immunoblotting with anti-VWF antibodies. ADAMTS-13 activity is taken to be absent in case ultra-large VWF multimers are still visible after proteolysis and present if the ultra-large multimers are not visible anymore after proteolysis. With the introduction of the FRETS-VWF73 assay, a more rapid method became available that facilitates quantitative measurement of ADAMTS-13 activity within 1 h [13]. A fluorescent signal is detected when the substrate comprising 73 amino acids of the VWF A2 domain is cleaved by ADAMTS-13 in the patient plasma. ADAMTS-13 activity is now expressed in terms of percentage compared with the activity found in normal pooled plasma. Such a rapid method, when proven valuable, can become of great importance for the adjustment of proper treatment of patients. We performed a prospective study in 79 patients with Coomb's negative hemolytic anemia and thrombocytopenia, where TTP was considered. Plasma samples were collected before the onset of plasma exchange and tested for the presence of ADAMTS-13 activity with both the proteolytic multimer assay and the FRETS-VWF73 assay. Plasma exchange was started independently of this result and the final diagnosis, TTP or an underlying disease causing thrombocytopenia and hemolytic anemia, was made by the treating physician upon hospital dismissal or death. Table 1 presents the results of the two ADAMTS-13 activity assays in the 79 patients that were considered TTP in the differential diagnosis stage. Upon hospital dismissal or death, 22 patients were diagnosed as primary TTP, five as pregnancy-related TTP, eight as HUS, 11 as TTP after bone marrow transplantation, four as sepsis, four as malignant hypertension, eight as haemolysis, elevated liver enzymes and low platelets (HELLP), seven as cancer, four as renal insufficiency and another six as other diseases. The diagnosis was set without prior knowledge of the ADAMTS-13 results. Twenty-four of the 27 patients (89%) that were diagnosed as primary TTP or pregnancy-related TTP did not show VWF proteolysis in the proteolytic multimer assay. In the FRETS-VWF73 assay, activity in these 24 patients was found to be 0%. Three primary TTP patients (11%), who had normal proteolysis in the proteolytic multimer assay, appeared to have activities varying from 53% to 75% in the FRETS-VWF73 assay. In the other 55 patients, where thrombocytopenia and hemolytic anemia were caused by the underlying diseases, normal VWF proteolysis was found in the proteolytic multimer assay. Here, the activity in the FRETS-VWF73 assay was found to be between 25% and 103%. One patient with sepsis and normal proteolysis in the proteolytic multimer assay had an activity of 3% in the FRETS-VWF73 assay. To summarize, we found that absence of ADAMTS-13 activity was 89% sensitive and 100% specific for TTP. This was concluded from both the labour-intensive proteolytic multimeric assay and the rapid FRETS-VWF73 assay, pointing at a 100% consistency between these two tests. In our opinion, the easy use and rapidity of FRETS-VWF73 have made it to a valuable and predictive tool in TTP diagnostics." @default.
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• W1969804753 date "2006-03-01" @default.
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• W1969804753 title "FRETS‐VWF73: a rapid and predictive tool for thrombotic thrombocytopenic purpura" @default.
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• W1969804753 doi "https://doi.org/10.1111/j.1538-7836.2005.01767.x" @default.
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