Essential (or primary) thrombocythemia (ET) (short version)
Compliance rules
1Summary
Essential (or primary) thrombocythemia (ET or PT) is a chronic myeloproliferative neoplasia (MPN) (formerly chronic myeloproliferative disease, CMPE) characterized by a persistent increase in platelet count. On the basis of molecular biological analyses (mutations in the JAK2, CALR, MPL genes), different subtypes can be distinguished. Typical disease complications are thrombosis and bleeding. Inhibitors of platelet aggregation and cytoreductive drugs are available for primary and secondary prevention of these complications. In a proportion of ET cases, the disease progresses to polycythaemia vera (PV), post-ET myelofibrosis or myelodysplastic syndrome (MDS), or acute leukemia.
2Therapy
The current treatment algorithm is depicted in Figure 1.
1Anagrelide is approved for the treatment of high-risk ET in the form of different preparations from different manufacturers, covering first- and second-line therapy in Germany, Austria and Switzerland.
2Pegylated interferon-alpha is only applicable as 'off label use'.
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10Active studies
11Systemic Therapy – Protocols
12Study results
13Certification Status
14Links
16Disclosures
Conflicts of interest can be found in the full German version of the guideline.
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