A case of primary cutaneous peripheral T‐cell lymphoma, not otherwise specified, with cytotoxic phenotype showing multiple ulcers on the entire body

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

Nature Communications ◽

10.1038/s41467-021-24037-4 ◽

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.

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An indolent nasal form of primary cutaneous CD8‐positive peripheral T‐cell lymphoma not otherwise specified successfully treated with local radiation therapy: A new subtype?

Dermatologic Therapy ◽

10.1111/dth.14887 ◽

2021 ◽

Vol 34 (2) ◽

Author(s):

Chiara Miano ◽

Matteo Megna ◽

Paola Di Caterino ◽

Emilio Berti ◽

Gabriella Fabbrocini ◽

...

Keyword(s):

Radiation Therapy ◽

T Cell ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Peripheral T Cell Lymphoma ◽

Not Otherwise Specified ◽

Local Radiation

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

The Journal of Dermatology ◽

10.1111/1346-8138.14029 ◽

2017 ◽

Vol 44 (12) ◽

pp. e359-e360 ◽

Cited By ~ 1

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

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

Case Reports in Oncology ◽

10.1159/000488236 ◽

2018 ◽

Vol 11 (1) ◽

pp. 212-215 ◽

Cited By ~ 1

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.

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