General background and pathophysiology Essential thrombocythemia (ET) belongs to a family of related disorders characterized by an uncontrolled cell growth, named myeloproliferative neoplasms (MPNs), also including polycythemia vera (PV) and primary myelofibrosis (PMF). In the World Health Organization (WHO) classification, the word 'neoplasm' was introduced instead of 'disorder' in order to underline the clonal character of the diseases. ET by far has the best prognosis of the three, with expected life duration close to normal. The phenotypes of the three related neoplasms differ considerably, even if there are similarities. In PV, the main characteristic is increased red cell mass and in ET increased platelet levels, whereas anemia is the main feature in myelofibrosis (MF). Thrombocytosis is a prerequisite for the diagnosis of ET, but may accompany both of the others, although more seldom. Transitions may occur: ET may develop into PV or MF, and PV may develop into MF. One school of thought believes in a pathophysiological continuum, starting from ET and ending in MF. However, this development is seen in a small minority of patients. Somewhat more common is the transition from ET directly to MF, but even this is a rare event, making it unlikely that the continuum theory may explain the basic pathophysiology.