title: Novel t(5;9)(q33;q22) fuses ITK to SYK in unspecified peripheral T-cell lymphoma.
abstract: Among peripheral T-cell lymphomas (PTCL), the heterogeneous category of unspecified PTCL represents the most common subtype. Nevertheless, recurrent chromosomal translocations are unknown in this aggressive type of lymphoma. Here we describe a novel t(5;9)(q33;q22) in unspecified PTCL. Molecular analyses delineated the breakpoints to ITK and SYK resulting in a previously undescribed expression of the Syk tyrosine kinase by Itk. ITK-SYK transcripts were detected in five of 30 (17%) unspecified PTCL, but not in cases of angioimmunoblastic T-cell lymphoma (n=9) and anaplastic lymphoma kinase-negative anaplastic large-cell lymphoma (n=7). In all five translocation-positive cases, the breakpoints were identical fusing the N-terminal pleckstrin homology domain and proline-rich region of ITK to the tyrosine kinase domain of SYK. Three of the five t(5;9)(q33;q22)+ unspecified PTCL shared a very similar histological pattern with predominant involvement of lymphoid follicles and the same CD3+CD5+CD4+bcl-6+CD10+ immunophenotype. These results demonstrate the presence of a recurrent t(5;9)(q33;q22) in a subset of unspecified PTCL, which may represent a novel distinct subgroup of PTCL.
authors.affiliation: Department of Pathology, Medical University of Vienna, Vienna, Austria. berthold.streubel@meduniwien.ac.at
authors.forename: B
authors.initials: B
authors.lastname: Streubel
authors.forename: U
authors.initials: U
authors.lastname: Vinatzer
authors.forename: M
authors.initials: M
authors.lastname: Willheim
authors.forename: M
authors.initials: M
authors.lastname: Raderer
authors.forename: A
authors.initials: A
authors.lastname: Chott
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D002895:Chromosomes, Human, Pair 5 / Q000235:genetics*; D002899:Chromosomes, Human, Pair 9 / Q000235:genetics*; D003001:Cloning, Molecular; D020732:Cytogenetic Analysis; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D017404:In Situ Hybridization, Fluorescence; D047908:Intracellular Signaling Peptides and Proteins / Q000235:genetics*; D008228:Lymphoma, Non-Hodgkin / Q000235:genetics* / Q000473:pathology; D016411:Lymphoma, T-Cell, Peripheral / Q000235:genetics* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D011505:Protein-Tyrosine Kinases / Q000235:genetics*; D020133:Reverse Transcriptase Polymerase Chain Reaction; D000072377:Syk Kinase; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology; D014158:Transcription, Genetic; D014178:Translocation, Genetic
chemical_list: D047908:Intracellular Signaling Peptides and Proteins; D015514:Oncogene Proteins, Fusion; D011505:Protein-Tyrosine Kinases; C000606896:SYK protein, human; D000072377:Syk Kinase; C080813:emt protein-tyrosine kinase