Incidence of acquired thrombotic thrombocytopenic purpura in Germany: a hospital level study
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(2019) 14:260
RESEARCH
Open Access
Incidence of acquired thrombotic thrombocytopenic purpura in Germany: a hospital level study Wolfgang Miesbach1* , Jan Menne2, Martin Bommer3, Ulf Schönermarck4, Thorsten Feldkamp5, Martin Nitschke6, Timm H. Westhoff7, Felix S. Seibert7, Rainer Woitas8, Rui Sousa9, Michael Wolf9, Stefan Walzer10 and Björn Schwander11
Abstract Background: Acquired thrombotic thrombocytopenic Purpura (aTTP) is a life-threatening ultra-orphan disease with a reported annual incidence between 1.5 and 6.0 cases per million in Europe and mainly affecting otherwise young and healthy adults aged 40 years on average. The goal of this study was to assess the incidence of aTTP in Germany. Methods: A systematic review was performed to determine the published evidence on the aTTP epidemiology in Germany. To obtain additional evidence on the proportion of aTTP cases within the national Thrombotic Microangiopathy (TMA) population a hospital-level study was performed, using a retrospective data collection approach. Diagnosis of aTTP was confirmed if ADAMTS13 level were < 10% and/or the medical records explicitly mentioned aTTP diagnosis. The aggregated hospital data were then projected to the national level using logistic regression techniques. Results: The systematic literature search did not provide incidence estimates of aTTP in Germany. Eight centers (≈27% of the top 30 TMA hospitals) delivered data according to a predefined data collection form. On average (year 2014–2016) a total number of 172 aTTP episodes per year was projected (95% confidence interval [95%CI]: 132–212). The majority were newly diagnosed aTTP cases (n = 121; 95%CI: 105–129), and 51 were recurrent aTTP cases (95%CI: 27–84). The average annual projected incidence (year 2014–2016) of aTTP episodes was 2.10 per million inhabitants in Germany (95%CI: 1.60–2.58). Conclusions: The determined annual incidence of newly diagnosed aTTP cases and the overall annual incidence of aTTP episodes in Germany confirm the ultra-orphan character of aTTP. An external validation against international registries (France, UK and USA) shows that our findings are quite comparable with those international incidence rates. Keywords: Epidemiology, Germany, Incidence, Thrombotic microangiopathy, Thrombotic thrombocytopenic purpura
Background Acquired thrombotic thrombocytopenic purpura (aTTP) is a potentially life-threatening thrombotic microangiopathy resulting from systemic microvascular thrombosis and leading to profound thrombocytopenia, hemolytic anemia, and organ failure of varying severity. Acquired TTP is caused by a severe deficiency of ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 * Correspondence: [email protected] 1 Universitätsklinikum Frankfurt, Medizinische Klinik II / Institut für Transfusionsmedizin und Immunhämatologie, Frankfurt, Germany Full list of author information is available at the end of the article
motif, member 13) due to the presence of inhibitory autoantibodies [1]. Decreased ADAMTS13 activity lead
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