Overview

NCI Definition: A chronic myeloproliferative neoplasm that involves primarily the megakaryocytic lineage. It is characterized by sustained thrombocytosis in the blood, increased numbers of large, mature megakaryocytes in the bone marrow, and episodes of thrombosis and/or hemorrhage. (WHO, 2008) [1]

Significant Genes in Essential Thrombocythemia

AFF1 +

CALR +

ELL +

IDH1 +

IDH2 +

JAK2 +

KMT2A +

MECOM +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

NRIP3 +

RPN1 +

Disease Details

Synonyms
Primary Thrombocytosis, ETC, Thrombocythemia, ET, Idiopathic Thrombocythemia, Essential Thrombocytemia, Primary Thrombocythemia, Essential Thrombocytosis
Parent(s)
Myeloproliferative Neoplasm
Children
Myelofibrosis Transformation in Essential Thrombocythemia
OncoTree Name
Essential Thrombocythemia
OncoTree Code
ET

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.