scholarly journals Sarcoidosis-Lymphoma Syndrome Associated with Folliculotropic Peripheral T Cell Lymphoma Not Otherwise Specified

2017 ◽  
Vol 10 (1) ◽  
pp. 372-376 ◽  
Author(s):  
Saaya Yoshida ◽  
Taku Fujimura ◽  
Yumi Kambayashi ◽  
Sadanori Furudate ◽  
Masato Mizuashi ◽  
...  

Sarcoidosis is occasionally accompanied by hematologic malignancies, including cutaneous T-cell lymphoma, called sarcoidosis-lymphoma syndrome. Although the mechanism underlying the induction of lymphomas is still unknown, understanding the immunological background of sarcoidosis could help explain the possible mechanisms of the induction of lymphomas. In this report, we describe a case of sarcoidosis-lymphoma syndrome associated with folliculotropic peripheral T cell lymphoma not otherwise specified, which caused dense infiltration of CD30+ CD163+ tumor-associated macrophages (TAMs) only in the lesional skin. Our present case might suggest the significance of TAMs in developing sarcoid-lymphoma syndrome.

2017 ◽  
Vol 10 (1) ◽  
pp. 328-332 ◽  
Author(s):  
Kayo Tanita ◽  
Taku Fujimura ◽  
Yota Sato ◽  
Takanori Hidaka ◽  
Sadanori Furudate ◽  
...  

Acquired ichthyosis (AI) is a reactive cutaneous manifestation that can be associated with malignant hematological disease, including cutaneous T-cell lymphoma (CTCL). Since it is difficult to distinguish AI from ichthyosiform mycosis fungoides, to select the treatment for CTCL with ichthyosis-like appearance and to evaluate its efficacy is sometimes challenging. In this report, we describe a case of primary cutaneous peripheral T-cell lymphoma not otherwise specified presenting AI successfully treated with oral bexarotene. In the present case, the administration of oral bexarotene was not only effective for lymphoma cells infiltrating ulcers and nodules, but it also eliminated AI.


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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