Primary cutaneous peripheral T-cell lymphoma not otherwise specified a rare and aggressive lymphoma

2018 ◽  
Vol 32 (10) ◽  
pp. e373-e376
Author(s):  
A. Pileri ◽  
C. Agostinelli ◽  
F. Fuligni ◽  
A. Broccoli ◽  
S. Gunnella ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Aggressive Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
Not Otherwise Specified

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.

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.

Case of peripheral T-cell lymphoma, not otherwise specified, presenting dyshidrosis-like eruptions as the first clinical manifestation

2017 ◽  
Vol 44 (12) ◽  
pp. e359-e360 ◽  
Author(s):  
Moeko Hotta ◽  
Teruki Yanagi ◽  
Tatsuro Sugai ◽  
Shinichi Nakazato ◽  
Kentaro Izumi ◽  
...  
Keyword(s):  
T Cell ◽  
Clinical Manifestation ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
Not Otherwise Specified

scholarly journals Successful Treatment of Advanced Primary Cutaneous Peripheral T-Cell Lymphoma with Oral Bexarotene Monotherapy

2018 ◽  
Vol 11 (1) ◽  
pp. 212-215 ◽  
Author(s):  
Yota Sato ◽  
Taku Fujimura ◽  
Yumi Kambayashi ◽  
Akira Hashimoto ◽  
Setsuya Aiba
Keyword(s):  
T Cell ◽  
Successful Treatment ◽  
Cell Lymphoma ◽  
Skin Lesions ◽  
Retinoid X Receptor ◽  
T Cell Lymphoma ◽  
Third Generation ◽  
Peripheral T Cell Lymphoma ◽  
Not Otherwise Specified ◽  
T Cell Lymphomas

Bexarotene is a third-generation retinoid X receptor-selective retinoid that is widely used for the early treatment of advanced-stage cutaneous T-cell lymphomas. In this report, we describe a case of successful treatment of advanced primary cutaneous peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) with oral bexarotene monotherapy. After the administration of oral bexarotene at a dose of 300 mg/m2/day, all skin lesions and lymph nodes regressed, and complete remission was achieved for 1 year. Our case suggested that bexarotene monotherapy could be one of the possible therapies for the treatment of primary cutaneous PTCL-NOS.

scholarly journals Identification of multiple subclones in peripheral T ‐cell lymphoma, not otherwise specified with genomic aberrations

2012 ◽  
Vol 1 (3) ◽  
pp. 289-294 ◽  
Author(s):  
Noriaki Yoshida ◽  
Akira Umino ◽  
Fang Liu ◽  
Kotaro Arita ◽  
Kennosuke Karube ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
Not Otherwise Specified ◽  
Genomic Aberrations
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