Ph‐like acute lymphoblastic leukemia with a novel PAX5‐KIDINS220 fusion transcript

Although “paired box 5” (PAX5)‐related fusion genes are well documented in childhood B‐cell precursor acute lymphoblastic leukemia (ALL), these types of fusion with the exception of PAX5‐JAK2 are rarely seen in patients with gene expression profiles similar to those of BCR‐ABL1 (Philadelphia)‐positi...

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Published in:Genes chromosomes & cancer 2017-04, Vol.56 (4), p.278-284
Main Authors: Sakamoto, Kenichi, Imamura, Toshihiko, Kanayama, Takuyo, Yano, Mio, Asai, Daisuke, Deguchi, Takao, Hashii, Yoshiko, Tanizawa, Akihiko, Ohshima, Yusei, Kiyokawa, Nobutaka, Horibe, Keizo, Sato, Atsushi
Format: Article
Language:English
Subjects:
Child
Humans
Male
Membrane Proteins - genetics
Nerve Tissue Proteins - genetics
Oncogene Proteins, Fusion - genetics
PAX5 Transcription Factor - genetics
Philadelphia Chromosome
Precursor Cell Lymphoblastic Leukemia-Lymphoma - genetics
Precursor Cell Lymphoblastic Leukemia-Lymphoma - pathology
Prognosis
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Summary:Although “paired box 5” (PAX5)‐related fusion genes are well documented in childhood B‐cell precursor acute lymphoblastic leukemia (ALL), these types of fusion with the exception of PAX5‐JAK2 are rarely seen in patients with gene expression profiles similar to those of BCR‐ABL1 (Philadelphia)‐positive ALL (Ph‐like ALL). We report a novel fusion of the genes PAX5 and “kinase D‐interacting substrate of 220 kDa” (KIDINS220, also known as ARMS) in a Ph‐like ALL. As PAX5 is a master regulator of B‐lymphocyte differentiation, PAX5 rearrangements induce a differentiation block in B lymphocytes. KIDINS220 is a mediator of multiple receptor signaling pathways, interacts with both T‐ and B‐cell receptors, and is necessary for sustained extracellular signal‐regulated kinase (ERK) signaling. Although functional studies are needed, the PAX5‐KIDINS220 fusion protein might not only inhibit wild‐type PAX5 function, but also promote sustained activation of the ERK signaling pathway through upregulation of KIDINS220. © 2016 Wiley Periodicals, Inc.
ISSN:1045-2257
1098-2264
DOI:10.1002/gcc.22433