Bleeding time and platelet function in essential thrombocythemia and other myeloproliferative syndromes

Bleeding time (BT) and platelet function tests have been widely used in patients with essential thrombocythemia (ET), with the aim to support diagnosis and to identify laboratory predictors of haemorrhagic and thrombotic complications. BT is significantly prolonged in 7-19% of ET patients and several functional abnormalities have been observed in platelet structure, biochemistry and survival. However, the attempt to relate these in vivo and in vitro platelet dysfunctions with diagnosis or clinical sequelae has been generally disappointing. Therefore, BT and platelet function tests are currently not recommended in the initial evaluation or during the follow-up of patients with ET, unless in the setting of a clinical or biological study. A noteworthy exception is represented by a subset of patients characterized by very high platelet count (> 1500 x 10(9)/L) and bleeding symptoms, who can have an acquired von Willebrand disease. In these cases, prolonged BT and abnormal multimeric pattern of von Willebrand factor are useful for diagnosing and monitoring this acquired hemorrhagic disease. BT and platelet function tests should be included in the baseline evaluation of ET patients enrolled in prospective clinical trials aiming assess their predictive role on clinical end-points.