Austrian recommendations for the management of essential thrombocythemia.
Essential thrombocythemia
Management recommendations
Myeloproliferative neoplasms
Risk stratification
Treatment
Journal
Wiener klinische Wochenschrift
ISSN: 1613-7671
Titre abrégé: Wien Klin Wochenschr
Pays: Austria
ID NLM: 21620870R
Informations de publication
Date de publication:
Jan 2021
Jan 2021
Historique:
received:
28
03
2020
accepted:
19
10
2020
pubmed:
21
11
2020
medline:
30
1
2021
entrez:
20
11
2020
Statut:
ppublish
Résumé
According to the World Health Organization (WHO) classification, essential (primary) thrombocythemia (ET) is one of several Bcr-Abl negative chronic myeloproliferative neoplasms (MPN). The classical term MPN covers the subcategories of MPN: ET, polycythemia vera (PV), primary myelofibrosis (PMF), and prefibrotic PMF (pPMF). ET is marked by clonal proliferation of hematopoietic stem cells, leading to a chronic overproduction of platelets. At the molecular level a JAK2 (Janus Kinase 2), calreticulin, or MPL mutation is found in the majority of patients. Typical ongoing complications of the disease include thrombosis and hemorrhage. Primary and secondary prevention of these complications can be achieved with platelet function inhibitors and various cytoreductive drugs including anagrelide, hydroxyurea and interferon. After a long follow up, in a minority of ET patients the disease transforms into post-ET myelofibrosis or secondary leukemia. Overall, life expectancy with ET is only slightly decreased.
Identifiants
pubmed: 33215234
doi: 10.1007/s00508-020-01761-3
pii: 10.1007/s00508-020-01761-3
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
52-61Commentaires et corrections
Type : CommentIn
Références
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