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• W2119049377 abstract "Background and Objectives The pathomechanism of thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS) is associated with a severe deficiency of ADAMTS13 and factor H. The aim of this study was to quantify the levels of ADAMTS13 and factor H in the pharmaceutically licensed plasma for transfusion, Octaplas®, and the universally applicable plasma, Uniplas (development product, working title). Furthermore, Octaplas® batches of blood groups A, B, O, AB, and plasmas derived from different sources were compared. Materials and Methods Twenty‐four Octaplas® and three Uniplas batches were selected for the study. ADAMTS13 activities were measured by fluorescence resonance energy transfer assay, ADAMTS13 antigen levels were quantified using enzyme‐linked immunosorbent assay test kit, while factor H antigen levels were detected using radial immunodiffusion (RID) methods. In addition, von Willebrand factor (vWF) multimeric analyses were performed. Results Both Octaplas®, produced from US and European plasma of different blood groups, and Uniplas contain ADAMTS13 antigen and activity levels as well as factor H concentrations at normal levels without significant differences. In addition, Octaplas® and Uniplas show a vWF multimeric pattern comparable to normal plasma. Conclusion The study revealed that Octaplas® and Uniplas contain normal levels of ADAMTS13 at low batch‐to‐batch variations. Therefore, both products can substitute the missing or neutralized protease activity in TTP patients and thereby limit vWF‐dependent (platelet‐related) thrombosis. In addition, both plasma products contain factor H at a physiological level, and, thus can be used efficiently in the treatment of aHUS patients, which have been shown to benefit from plasma administration." @default.
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• W2119049377 date "2007-02-19" @default.
• W2119049377 modified "2023-10-11" @default.
• W2119049377 title "Normal levels of ADAMTS13 and factor H are present in the pharmaceutically licensed plasma for transfusion (Octaplas®) and in the universally applicable plasma (Uniplas) in development" @default.
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• W2119049377 doi "https://doi.org/10.1111/j.1423-0410.2006.00884.x" @default.
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