PubMed · 1198529
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J A van Mourik, W T La Bruyére, I A Mochtar. 1975-11-15. Proceedings: Subunit structure of human factor VIII.. https://pubmed.ncbi.nlm.nih.gov/1198529/
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This report presents seven children with congenital thrombotic thrombocytopenic purpura (TTP). Six had a history of severe neonatal unconjugated hyperbilirubinaemia and thrombocytopenia. The seventh child had no neonatal problems but has suffered three episodes of acute TTP. The subsequent clinical course of the children varied. Five had a relapsing-remitting course and one had chronic microangiopathic haemolytic anaemia. The five oldest children initially received plasma infusions but, because of viral safety issues and easier administration, they now receive intermediate purity US-sourced plasma-derived factor VIII concentrate: BPL 8Y. Effective prophylaxis and treatment is possible in congenital TTP using BPL 8Y.
Quantification of the bleeding severity in von Willebrand disease (VWD) by use of a bleeding score (BS) could be a useful tool toward a more objective diagnosis. The BS is computed as the sum of arbitrary grades of bleeding severity for several hemorrhagic symptoms, and in normal subjects is nearly always lower than 3. In the diagnosis of type 1 VWD, the use of the BS provided a high specificity (99.1%) and a satisfactory sensitivity (64.2%) in a multicenter, retrospective study. The BS is strongly related to all available measurements of von Willebrand factor activity, including the PFA-100 closure time. The BS could be used to assess the influence on bleeding symptoms of other biological factors in VWD. Therefore, BS represent a promising clinical tool for the analysis of bleeding in VWD, although further validation is warranted before an extensive use in clinical practice could be purported.