title: C-MYC rearrangement may induce an aggressive phenotype in anaplastic lymphoma kinase positive anaplastic large cell lymphoma: Identification of a novel fusion gene ALO17/C-MYC.
abstract: Anaplastic lymphoma kinase (ALK) positive anaplastic large cell lymphoma (ALCL) is usually associated with a favorable prognosis. We describe an 11-year-old girl patient with ALK positive ALCL bearing t(2;5)(p23;q35) and t(8;17)(q24;q25) translocations who had an aggressive clinical course despite various combinations of intensive chemotherapy. Southern blot analysis identified C-MYC rearrangement. Immunohistochemistry and Northern and Western blot analyses revealed cmyc overexpression. A new fusion between ALO17 (ALK lymphoma oligomerization partner on chromosome 17) and C-MYC was identified by the 50-rapid amplification of cDNA ends. This new fusion may have possibly provoked the poor prognosis in this patient with ALK positive ALCL, and C-MYC rearrangement may indicate poor prognosis in ALCL.
authors.affiliation: Division of Pediatrics, Department of Reproductive and Developmental Medicine, University of Miyazaki, Japan. moritake@fc.miyazaki-u.ac.jp
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Moritake
authors.forename: Hidemi
authors.initials: H
authors.lastname: Shimonodan
authors.forename: Kousuke
authors.initials: K
authors.lastname: Marutsuka
authors.forename: Sachiyo
authors.initials: S
authors.lastname: Kamimura
authors.forename: Hitomi
authors.initials: H
authors.lastname: Kojima
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Nunoi
mesh_terms: D000077548:Anaplastic Lymphoma Kinase; D002648:Child; D002886:Chromosomes, Human, Pair 17; D005260:Female; D015321:Gene Rearrangement; D016259:Genes, myc; D006801:Humans; D017728:Lymphoma, Large-Cell, Anaplastic / Q000201:enzymology / Q000235:genetics* / Q000473:pathology; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D010641:Phenotype; D011505:Protein-Tyrosine Kinases / Q000096:biosynthesis / Q000235:genetics*; D020794:Receptor Protein-Tyrosine Kinases
chemical_list: D015514:Oncogene Proteins, Fusion; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D011505:Protein-Tyrosine Kinases; D020794:Receptor Protein-Tyrosine Kinases