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Editorial
. 2017 Oct 26;8(57):96466-96467.
doi: 10.18632/oncotarget.22098. eCollection 2017 Nov 14.

The enigmatic role(s) of P2RY8-CRLF2

Renate Panzer-Grümayer  1 , Stefan Köhrer  1 , Oskar A Haas  1
Affiliations
Editorial

The enigmatic role(s) of P2RY8-CRLF2

Renate Panzer-Grümayer et al. Oncotarget. .
No abstract available

Keywords: CRLF2-fusion; IKZF1 alterations; P2RY8-CRLF2; childhood ALL; clonal evolution.

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Figures

Figure 1
Figure 1. Model for the evolution of P2RY8-CRLF2-positive leukemia and selection of relapse clones
Leukemia-initiating (founder) alterations generally occur in a hematopoietic stem/progenitor cell – and are present in all progenies, while the ensuing RAG-mediated microdeletions evolve only later during early B cell differentiation. Microdeletions affect genes critical for B cell differentiation and tumor suppression. JAK/STAT and/or RTK/Ras pathway activating alterations may emerge during the same time span, are usually subclonal and do not outcompete each other at initial presentation of leukemia. Chemotherapy (Tx) then selects for resistant clones (e.g. IKZF1 mutant), which are either already present in the dominant clone, as depicted here, or in a small subclone of the initial leukemia. IKZF1 mutant clones reside in the bone marrow (BM) niche and are there protected from chemotherapy. Only acquisition of proliferation-driving mutations leads to manifestations of the relapse. The model is based on the most striking results of NGS data from our latest study [4]. Each color in the figure represents a particular alteration in a cell and its descendent cells, in which further mutations occur (color code at left bottom of the graph).
See this image and copyright information in PMC

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