OBJECTIVES: The antibody-mediated, prothrombotic heparin-induced thrombocytopenia type II (HIT-II) is a life-threatening disease with high thrombosis risk of 38-76% and up to 20% mortality. Pulmonary embolism occurs in up to 40% of patients, and amputations are necessary in up to 15%. The objective was to evaluate frequency and cost of HIT-II in Germany. METHODS: Systematic literature searches regarding epidemiology and cost of HIT-II were conducted until end of 2000 with Medical Subject Heading terms “incidence”, “epidemiology”, “risk”, and “cost” each in combination with “heparin-induced thrombocytopenia”. German secondary data were obtained by desktop research from the German Federal Statistical Office and a German university hospital. RESULTS: Literature search yielded eleven relevant publications selected by successive title, abstract and whole publication screening from a total of 1225 hits. Published incidence for HIT-II in Germany was 0.039% for in-hospital patients, and average additional costs per patient amounted to €9004 (Wilke et al., J Thromb Haemost, 2009). Data from the German Federal Statistical Office for 2009 show an incidence of 0.05% for patients with secondary diagnosis HIT-II, ICD-10 Code D69.53, corresponding to 8,585 cases (age peak 65-85 years) with an average prolongation of hospital stay by 18 days. The frequency of documented HIT-II as secondary diagnosis increased since 2005 by 60.4% (2005: 5353; 2006: 6263; 2007: 7177; 2008: 7454 cases). Estimated additional costs generated by HIT-II in Germany in 2005 amount to 48 million euro, and in 2009 to a minimum of 77 million euro. CONCLUSIONS: Cost and burden of HIT-II are considerable. Due to the demographic development to be expected in Germany during the next decades in combination with the age peak of the disease a further increase in HIT-II cases has to be anticipated. Data are limited. Further epidemiological research and analysis of burden of disease from several perspectives are needed.