scholarly journals Refractory Septic Shock due to Underlying Immunocompromised Disease: A Case of Fatal Peripheral T-Cell Lymphoma Not Otherwise Specified in a Young Hispanic Woman

2018 ◽  
Vol 11 (2) ◽  
pp. 404-411
Author(s):  
Kishore Kumar ◽  
Muhammad Saad ◽  
Chukwunonso Chime ◽  
Kanthi Badipatla ◽  
Hassan Tariq ◽  
...  

Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) is known as an unclassified group of non-Hodgkin lymphomas. PTCL-NOS is a subtype of peripheral T-cell lymphoma that accounts for 3% of all lymphomas in the United States. PTCL-NOS commonly presents as a disseminated disease, B symptoms (fever, night sweats, and weight loss), generalized lymphadenopathy, bone marrow infiltration, and extranodal involvement. Other rare and possible manifestations include peripheral eosinophilia, pruritus, or hemophagocytic syndrome. This type of lymphoma is aggressive in nature and carries a high mortality rate. There is no established standard-of-care treatment protocol for PTCL-NOS, several chemotherapy drug combinations are frequently used as a frontline treatment with an overall 5-year survival rate of 25%. We present a case of a 35-year old Hispanic woman who initially presented with a chief complaint of bilateral lower extremity swelling due to hypoalbuminemia and new-onset hyperkeratotic skin of the palms and soles.

2020 ◽  
Vol 2020 ◽  
Author(s):  
MOUNIA BENDARI ◽  
Wafaa Matrane ◽  
Maryam Qachouh ◽  
Asmaa Quessar ◽  
Nisrine Khoubila

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.


2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Koen Debackere ◽  
Lukas Marcelis ◽  
Sofie Demeyer ◽  
Marlies Vanden Bempt ◽  
Nicole Mentens ◽  
...  

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.


2017 ◽  
Vol 44 (12) ◽  
pp. e359-e360 ◽  
Author(s):  
Moeko Hotta ◽  
Teruki Yanagi ◽  
Tatsuro Sugai ◽  
Shinichi Nakazato ◽  
Kentaro Izumi ◽  
...  

2018 ◽  
Vol 11 (1) ◽  
pp. 212-215 ◽  
Author(s):  
Yota Sato ◽  
Taku Fujimura ◽  
Yumi Kambayashi ◽  
Akira Hashimoto ◽  
Setsuya Aiba

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.


2018 ◽  
Vol 32 (10) ◽  
pp. e373-e376
Author(s):  
A. Pileri ◽  
C. Agostinelli ◽  
F. Fuligni ◽  
A. Broccoli ◽  
S. Gunnella ◽  
...  

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