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Essential (or primary) thrombocythemia (ET) (short version)

Date of document August 2021
This is the current valid version of the document

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.

Figure 1: Treatment algorithm in patients with essential thrombocythemia (ET), based on the conventional score 
curative treatment intent; non-curative treatment intent;
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

15Authors' Affiliations

Prof. Dr. med. Petro E. Petrides
Hämatologisch-Onkologische Schwerpunktpraxis
am Isartor
Zweibrückenstr. 2
80331 München
Prof. Dr. med. Gabriela M. Baerlocher
Universität Bern
Labor für Hämatopoiese und Molekulare Genetik
Murtenstrasse 40
CH-3008 Bern
Prof. Dr. med. Konstanze Döhner
Universitätsklinikum Ulm
Innere Medizin III
Albert-Einstein-Allee 23
89081 Ulm
Prof. Dr. med. Heinz Gisslinger
Medizinische Universität in Wien
Universitätsklinik f.Innere Medizin I
Klinische Abteilung für Hämatologie und Hämostaseologie
Währinger Gürtel 18-20
A-1090 Wien
Prof. Dr. med. Martin Grießhammer
Johannes Wesling Klinikum Minden
Klinik für Hämatologie / Onkologie
Hans-Nolte-Str. 1
32429 Minden
Univ.-Prof. Dr. med. Steffen Koschmieder
Uniklinik RWTH Aachen
Med. Klinik IV
Hämatologie, Onkologie, Hämostaseologie & SZT
Pauwelsstr. 30
52074 Aachen
PD Dr. med. Juliana Schwaab
Universitätsmedizin Mannheim
III. Medizinische Klinik
Hämatologie und Internistische Onkologie
Theodor-Kulzer-Ufer 1-3
68167 Mannheim
Prof. Dr. med. Eva Lengfelder
Universitätsklinikum Mannheim
Medizinische Fakultät Mannheim d. Uni Heidelberg
III. Medizinische Klinik
Theodor-Kutzer-Ufer 1-3
68167 Mannheim

16Disclosures

Conflicts of interest can be found in the full German version of the guideline.

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