scholarly journals FYN-TRAF3IP2 and KHDRBS1-LCK hijack T cell receptor signaling in peripheral T cell lymphoma, not otherwise specified

2021 ◽  
Author(s):  
Koen Debackere ◽  
Lukas Marcelis ◽  
Sofie Demeyer ◽  
Marlies Vanden Bempt ◽  
Nicole Mentens ◽  
...  
Keyword(s):  
T Cell ◽  
T Cell Receptor ◽  
Cell Lymphoma ◽  
Ex Vivo ◽  
Cell Receptor ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
In Vivo Experiments ◽  
Not Otherwise Specified

Abstract Peripheral T cell lymphoma (PTCL) is a heterogeneous group of non-Hodgkin lymphomas with poor prognosis. Up to 30% of PTCL lack distinctive features and are classified as PTCL, not otherwise specified (PTCL-NOS). To further improve our understanding of the genetic landscape and biology of PTCL-NOS, we performed RNA-sequencing of 18 cases and validated results in an independent cohort of 37 PTCL cases. We identified FYN-TRAF3IP2, KHDRBS1-LCK and SIN3A-FOXO1 as new in-frame fusion transcripts, with FYN-TRAF3IP2 as a recurrent fusion detected in 8 of 55 cases. Using ex vivo and in vivo experiments, we demonstrate that FYN-TRAF3IP2 and KHDRBS1-LCK activate signaling pathways downstream of the T cell receptor (TCR) complex and confer therapeutic vulnerability to clinically available drugs.

scholarly journals Fusion transcripts FYN-TRAF3IP2 and KHDRBS1-LCK hijack T cell receptor signaling in peripheral T-cell lymphoma, not otherwise specified

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Koen Debackere ◽  
Lukas Marcelis ◽  
Sofie Demeyer ◽  
Marlies Vanden Bempt ◽  
Nicole Mentens ◽  
...  
Keyword(s):  
T Cell ◽  
T Cell Receptor ◽  
Cell Lymphoma ◽  
Ex Vivo ◽  
Cell Receptor ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
Fusion Transcripts ◽  
Not Otherwise Specified

AbstractPeripheral T-cell lymphoma (PTCL) is a heterogeneous group of non-Hodgkin lymphomas with poor prognosis. Up to 30% of PTCL lack distinctive features and are classified as PTCL, not otherwise specified (PTCL-NOS). To further improve our understanding of the genetic landscape and biology of PTCL-NOS, we perform RNA-sequencing of 18 cases and validate results in an independent cohort of 37 PTCL cases. We identify FYN-TRAF3IP2, KHDRBS1-LCK and SIN3A-FOXO1 as new in-frame fusion transcripts, with FYN-TRAF3IP2 as a recurrent fusion detected in 8 of 55 cases. Using ex vivo and in vivo experiments, we demonstrate that FYN-TRAF3IP2 and KHDRBS1-LCK activate signaling pathways downstream of the T cell receptor (TCR) complex and confer therapeutic vulnerability to clinically available drugs.

The fusion kinase ITK-SYK mimics a T cell receptor signal and drives oncogenesis in conditional mouse models of peripheral T cell lymphoma

2010 ◽  
Vol 189 (4) ◽  
pp. i10-i10
Author(s):  
Konstanze Pechloff ◽  
Julian Holch ◽  
Uta Ferch ◽  
Marc Schweneker ◽  
Kristina Brunner ◽  
...  
Keyword(s):  
T Cell ◽  
T Cell Receptor ◽  
Mouse Models ◽  
Cell Lymphoma ◽  
Cell Receptor ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
Receptor Signal

scholarly journals Self-Reactive T Cell Receptor-Reactive CD8+ T Cells Inhibit T Cell Lymphoma Growth In Vivo

2004 ◽  
Vol 173 (11) ◽  
pp. 7062-7069 ◽  
Author(s):  
Marie Gonthier ◽  
Régine Llobera ◽  
Jacques Arnaud ◽  
Bent Rubin
Keyword(s):  
T Cells ◽  
T Cell ◽  
T Cell Receptor ◽  
Cd8 T Cells ◽  
Cell Lymphoma ◽  
Cell Receptor ◽  
T Cell Lymphoma

T-cell receptor-silent peripheral T-cell lymphoma complicated with hemophagocytic lymphohystiocytosis

2021 ◽  
Author(s):  
Koya Ono ◽  
Yasushi Onishi ◽  
Koichi Onodera ◽  
Daigo Michimata ◽  
Eijiro Furukawa ◽  
...  
Keyword(s):  
T Cell ◽  
T Cell Receptor ◽  
Cell Lymphoma ◽  
Cell Receptor ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma

Classical Hodgkin Lymphoma With Aberrant CD8 Expression: A Clinicopathologic Study of Five Cases

2018 ◽  
Vol 27 (2) ◽  
pp. 166-173
Author(s):  
Neda Mirzamani ◽  
Xinmin Zhang ◽  
Judith Brody ◽  
Silvia G. Spitzer ◽  
Filiz Sen ◽  
...  
Keyword(s):  
T Cell ◽  
Hodgkin Lymphoma ◽  
Tumor Cells ◽  
T Cell Receptor ◽  
Cell Lymphoma ◽  
Cell Receptor ◽  
T Cell Lymphoma ◽  
Classical Hodgkin Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
T Cell Receptor Genes

Hodgkin/Reed-Sternberg (HRS) cells of classical Hodgkin lymphoma (CHL) are of B-cell origin. In a small number of CHL cases, the tumor cells can express T-cell antigens. CD8 expression in this setting is extremely rare. We identified 5 cases of CHL with aberrant CD8 expression from our database. The patients included 3 men and 2 women with a median age of 33 years (range = 20-59 years). All the patients initially presented with lymphadenopathy and variable number of RS cells. Two cases were classified as mixed cellularity type that showed prominent vascular proliferation mimicking peripheral T-cell lymphoma. Two cases represented nodular sclerosis type. The tumor cells in all cases were positive for CD8 and negative for CD2, CD3, CD4, and CD7 and carried germline T-cell receptor genes. Molecular studies revealed T-cell receptor genes to be in germline configuration in 4 cases with available information. Given the morphologic overlap with peripheral T-cell lymphoma and the rarity of this type of CHL, identifying more cases will help our better understanding of this entity.

Primary T-Cell Lymphoma of The Testis Presenting In A Young Adult

2020 ◽  
Vol 2020 ◽  
Author(s):  
MOUNIA BENDARI ◽  
Wafaa Matrane ◽  
Maryam Qachouh ◽  
Asmaa Quessar ◽  
Nisrine Khoubila
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Clinical Stage ◽  
T Cell Lymphoma ◽  
Second Line ◽  
Peripheral T Cell Lymphoma ◽  
Not Otherwise Specified ◽  
Ann Arbor ◽  
Prophylactic Chemotherapy ◽  
Line Chemotherapy

We report the case of a 40-year-old male presented with a painless right testicular swelling. Right radical orchidectomy was performed. The pathological diagnosis was peripheral T-Cell lymphoma-not otherwise specified (PTCL-NOS). According to Ann Arbor staging, the initial clinical stage was IEa. Treating him with four courses of the CHOEP protocol and intrathecal prophylactic chemotherapy was unsuccessful; with the appearance of orbital infiltration and a loco-regional extension. Although the patient started a second line chemotherapy, he unfortunately succumbed to death.

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