Overview

NCI Definition: Progression of polycythemia vera characterized by decreased erythropoiesis and granulopoiesis, reticulin and collagen fibrosis of the bone marrow, leucoerythroblastic reaction in the peripheral blood, poikilocytosis, tear-drop shaped red blood cells, and splenomegaly. [1]

Polycythemia vera, post-polycythemic myelofibrosis phases most frequently harbor alterations in JAK2, TET2, EZH2, ASXL1, and SETD1B [2].

Most Commonly Altered Genes in Polycythemia Vera, Post-Polycythemic Myelofibrosis Phase

JAK2 V617F, JAK2 Mutation, JAK2 Exon 14 Mutation, TET2 Mutation, and TET2fs are the most common alterations in polycythemia vera, post-polycythemic myelofibrosis phase [2].

Top Alterations in Polycythemia Vera, Post-Polycythemic Myelofibrosis Phase

Significant Genes in Polycythemia Vera, Post-Polycythemic Myelofibrosis Phase

AFF1 +

ELL +

JAK2 +

KMT2A +

MECOM +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

RPN1 +

Disease Details

Synonyms
Polycythemia Vera, "Spent" Phase, Post-PV MF, Polycythemia Vera, Post-Polycythemic Myelofibrosis and Myeloid Metaplasia Stage, Post-polycythemia Vera Myelofibrosis, Spent Phase and Post-Polycythemic Myelofibrosis
Parent(s)
Secondary Myelofibrosis
OncoTree Name
Polycythaemia Vera Myelofibrosis
OncoTree Code
PVMF

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.