Heparin-induced thrombocytopenia (HIT) and HIT thrombosis syndrome (HITTS) are immune-mediated complications of clinical use of unfractionated heparin (UFH). The antibody/antigen complex is composed of heparin and platelet factor 4. This complex not only activates platelets but also the clotting system leading to thrombin generation. This explains the thrombosing tendency of these patients, and venous and arterial thromboembolisms are encountered with a morbidity and mortality of about 25-37%. The incidence of HIT is about 3% when UFH is administered therapeutically. The diagnosis is at this time based on clinical observations, especially a sudden, unexplained drop in platelet counts without other reasons. Laboratory tests can be used to confirm the clinical diagnosis, but none of the available tests is 100% reliable. There is no test that will predict HIT and no test that will signal the development of HITTS. Treatment consists of discontinuation of UFH in any form and anticoagulation with danaparoid or r-hirudin, if needed. The use of low molecular weight heparins instead of UFH could largely (not totally) alleviate the problem.