Survival Advantage With the Addition of Radiation Therapy to Chemotherapy in Early Stage Peripheral T-Cell Lymphoma, Not Otherwise Specified

We present the extraordinary case of a 72-year-old man with a history of primary cutaneous peripheral T-cell lymphoma not otherwise specified (pcPTCL-NOS) previously controlled with topical agents who developed tumours in a sporotrichoid pattern. Culture of the tumours was negative, and histopathology showed findings consistent with recurrent pcPTCL. The tumours were successfully treated with localised radiation therapy. Sporotrichoid lesions are an extremely rare and atypical presentation of cutaneous lymphoma, with only 2 other cases reported in the literature. Our case reinforces the need to include cutaneous lymphoma in the differential diagnosis of nodules on the extremities spreading in a sporotrichoid pattern. Clinical recognition of this atypical presentation of cutaneous lymphoma allows for prompt, effective treatment, which might include localised radiation therapy.

Download Full-text

Peripheral T-Cell Lymphoma not otherwise specified with oral manifestation: A Case Report.

Journal Of Oral Research ◽

10.17126/joralres.2020.098 ◽

2020 ◽

Vol 9 (6) ◽

pp. 516-521

Author(s):

Alvaro Vigouroux ◽

◽

Felipe Lillo ◽

Francisca Donoso ◽

◽

...

Keyword(s):

T Cell ◽

Cell Lymphoma ◽

Early Stage ◽

T Cell Lymphoma ◽

Patient Treatment ◽

Oral Lesions ◽

Peripheral T Cell Lymphoma ◽

Rare Type ◽

Oral Manifestation ◽

Not Otherwise Specified

Peripheral T-Cell Lymphoma Not Otherwise Specified it is a rare type of Non-Hodgkin t-cells malignant tumor whose oral manifestations are difficult to diagnose. A case of a 48-year-old male with a hemi-maxillary lesion histological and immunohistochemically compatible with Peripheral T-Cell Lymphoma not otherwise specified is presented. A case of a 48-year-old male with a hemi-maxillary lesion histological and immunohistochemically compatible with Peripheral T-Cell Lymphoma not otherwise specified is presented. The patient treatment consisted of chemotherapy, but after the second cycle, died from immunosuppressive complications. Early stage diagnosis of oral lesions is imperative to avoid aggressive treatment and low overall survival rate of such pathologies.

Download Full-text

Chemotherapy Plus Radiotherapy Versus Chemotherapy Alone for Patients With Peripheral T-Cell Lymphoma, Not Otherwise Specified

Frontiers in Oncology ◽

10.3389/fonc.2021.607145 ◽

2021 ◽

Vol 11 ◽

Author(s):

Zegeng Chen ◽

He Huang ◽

Xiaoqian Li ◽

Xiaojie Fang ◽

Zhao Wang ◽

...

Keyword(s):

T Cell ◽

Survival Benefit ◽

Validation Cohort ◽

Cell Lymphoma ◽

Early Stage ◽

T Cell Lymphoma ◽

Advanced Stage ◽

Peripheral T Cell Lymphoma ◽

Not Otherwise Specified ◽

Stage Disease

PurposePeripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) is a clinically and biologically heterogeneous disease with poor prognosis. As the role of radiation therapy (RT) is still unclear, we carried out this study to evaluate the potential efficacy of RT in PTCL-NOS.MethodsPatients diagnosed with PTCL-NOS between 2000 and 2016 were identified from the Surveillance, Epidemiology, and End Results (SEER) database. Propensity score matching was used to balance the characteristics between patients who received radiotherapy and those who did not receive radiotherapy. In addition, we validated the findings in an external validation cohort retrospectively recruited from two high-capacity cancer center in China between 2006 and 2016. Kaplan-Meier curves and Cox regression models were used for survival analysis.ResultsOf the 2,768 patients with chemotherapy records in the SEER cohort, 27.6% of 844 patients with early-stage disease and 6.8% of 1,924 patients with advanced-stage disease received RT. The application of RT was significantly associated with an improvement in overall survival (5-year OS rate 58.5 versus 35.1%, P <0.001) and disease-specific survival (5-year DSS rate 66.3 versus 44.0%, P <0.001) in the early-stage subgroup, while no apparent survival benefit of adding RT was identified in patients with advanced-stage disease (5-year OS rate 28.7 versus 24.4%, P = 0.089; 5-year DSS rate 32.9 versus 31.3%, P = 0.223). After adjustment, a matched cohort of 1,044 patients (348 in the RT combined with CT group and 696 in the CT alone group) was created. And RT was still significantly associated with a survival benefit in the early-stage subset, but not in the advanced-stage disease group. In the validation cohort with more comprehensive data, RT also significantly improved the survival of early-stage PTCL-NOS patients.ConclusionAdding RT was associated with significant improvement in survival in early-stage PTCL-NOS, but the survival benefit of RT was not obvious in advanced-stage disease. The incorporation of RT for treatment in early-stage PTCL-NOS should be highly considered. Further prospective studies with more comprehensive data are needed to evaluate the effectiveness and toxicity of RT in PTCL-NOS.

Download Full-text

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

Health Sciences ◽

10.15342/hs.2020.214 ◽

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.

Download Full-text

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.

Download Full-text

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

Download Full-text

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

Journal of Cutaneous Pathology ◽

10.1111/cup.13386 ◽

2018 ◽

Cited By ~ 3

Author(s):

Koya Obara ◽

Sumiyuki Mii ◽

Yasuyuki Amoh

Keyword(s):

T Cell ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Entire Body ◽

Peripheral T Cell Lymphoma ◽

Not Otherwise Specified ◽

Multiple Ulcers

Download Full-text

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.

Download Full-text