Austrian recommendations for the management of essential thrombocythemia.
Wien Klin Wochenschr
; 133(1-2): 52-61, 2021 Jan.
Article
em En
| MEDLINE
| ID: mdl-33215234
ABSTRACT
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.
Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Policitemia Vera
/
Mielofibrose Primária
/
Trombocitemia Essencial
/
Transtornos Mieloproliferativos
Tipo de estudo:
Diagnostic_studies
Limite:
Humans
País/Região como assunto:
Europa
Idioma:
En
Revista:
Wien Klin Wochenschr
Ano de publicação:
2021
Tipo de documento:
Article
País de afiliação:
Áustria