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.

Peripheral T-cell lymphomas unspecified presenting in the skin: analysis of prognostic factors in a group of 82 patients

Blood ◽  
2003 ◽  
Vol 102 (6) ◽  
pp. 2213-2219 ◽  
Author(s):  
Marcel W. Bekkenk ◽  
Maarten H. Vermeer ◽  
Patty M. Jansen ◽  
Ariënne M. W. van Marion ◽  
Marijke R. Canninga-van Dijk ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Size ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Skin Lesions ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
T Cell Lymphomas ◽  
Nor Phenotype ◽  
Peripheral T Cell Lymphomas

Abstract In the present study the clinicopathologic and immunophenotypic features of 82 patients with a CD30– peripheral T-cell lymphoma, unspecified, presenting in the skin were evaluated. The purpose of this study was to find out whether subdivision of these lymphomas on the basis of cell size, phenotype, or presentation with only skin lesions is clinically relevant. The study group included 46 primary cutaneous CD30– large cell lymphomas and 17 small/medium-sized T-cell lymphomas as well as 17 peripheral T-cell lymphomas with both skin and extracutaneous disease at the time of diagnosis. Patients with primary cutaneous small- or medium-sized T-cell lymphomas had a significantly better prognosis (5-year-overall survival, 45%) than patients with primary cutaneous CD30– large T-cell lymphomas (12%) and patients presenting with concurrent extracutaneous disease (12%). The favorable prognosis in this group with primary cutaneous small- or medium-sized T-cell lymphomas was particularly found in patients presenting with localized skin lesions expressing a CD3+CD4+CD8– phenotype. In the primary cutaneous T-cell lymphoma (CTCL) group and in the concurrent group, neither extent of skin lesions nor phenotype had any effect on survival. Our results indicate that peripheral T-cell lymphomas, unspecified, presenting in the skin have an unfavorable prognosis, irrespective of the presence or absence of extracutaneous disease at the time of diagnosis, cell size, and expression of a CD4+ or CD8+ phenotype. The only exception was a group of primary cutaneous small- or medium-sized pleomorphic CTCLs with a CD3+CD4+CD8– phenotype and presenting with localized skin lesions.

scholarly journals Primary T-cell Non-Hodgkin’s Lymphoma Presenting as a case of Testicular Mass

2016 ◽  
Vol 11 (1) ◽  
pp. 89-92 ◽  
Author(s):  
SM Abu Horaira ◽  
SM Badruddoza ◽  
Mahbubul Alam ◽  
SK Jaynul Islam ◽  
Md Kabiruzzaman Shah
Keyword(s):  
T Cell ◽  
World Literature ◽  
Cell Lymphoma ◽  
Armed Forces ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
Medical College ◽  
Not Otherwise Specified ◽  
The Poor ◽  
T Cell Lymphomas

Introduction: T-cell lymphoma is a very rare tumor. Only a few cases have been reported in world literature. It involves the testis infrequently, which deserves special attention because of the poor prognosis and the need to make an appropriate diagnosis, which can lead to a better therapeutic strategy.Case presentation: A 34-year-old man presented with left testicular swelling for past four months. The swelling was painless, hard and rubbery. Testicular ultrasound showed diffuse increase in size of the testicle, with alteration in its echogenicity. FNAC was done and the diagnosis was Non-Hodgkin’s lymphomas. The patient underwent orchidectomy and basing on histopathological and immunohistochemical tests, a peripheral T-cell lymphoma, not otherwise specified was diagnosed.Conclusion: Testicular peripheral T-cell lymphomas are rare and aggressive cancers which is clinically different from seminoma and other non-neoplastic conditions.Journal of Armed Forces Medical College Bangladesh Vol.11(1) 2015: 89-92

scholarly journals A rare case of peripheral T cell lymphoma, not otherwise specified with cutaneous involvement and diffuse systemic metastasis

2021 ◽  
Vol 7 (3) ◽  
pp. 476
Author(s):  
Pandharinath K. Khade ◽  
Agni K. Bose ◽  
Vidya D. Kharkar
Keyword(s):  
Gastrointestinal Tract ◽  
T Cell ◽  
Poor Prognosis ◽  
Cell Lymphoma ◽  
Palliative Radiotherapy ◽  
T Cell Lymphoma ◽  
Lower Limbs ◽  
Peripheral T Cell Lymphoma ◽  
Not Otherwise Specified ◽  
T Cell Lymphomas

<p class="abstract">Peripheral T-cell lymphoma (PTCL), a subdivision of T-cell non-Hodgkin lymphomas (NHLs), is rare and different from the more common cutaneous T-cell lymphomas. PTCL is a diverse group of disorders and carries a poor prognosis. Peripheral T cell lymphoma not otherwise specified (PTCL-NOS) is the most common and aggressive disorder out of the group, which involves the lymph nodes followed by the skin, liver, and gastrointestinal tract. We hereby report a case of a 60 years old female who presented to us with complaints of multiple painful dark-coloured nodular lesions and indurated plaques over bilateral upper limbs, lower limbs and trunk, for 3 months. After detail laboratory investigations, histopathological and immunohistochemistry which was positive for CD30 antigen we confirmed the diagnosis as PTCL-NOS.  Radiological imaging showed involvement of gastrointestinal tract, adrenal gland and vertebrae. After thorough workup the patient was started on palliative radiotherapy, unfortunately she succumbed to death. In conclusion, PTCL-NOS with CD30 positivity is rare and aggressive with a poor prognosis, it is important to identify such cases and work in conjugation with an oncologist for proper management.</p>

scholarly journals Unspecified skin peripheral T-cell lymphoma: diagnostic difficulties

2020 ◽  
Vol 15 (3) ◽  
pp. 63-66
Author(s):  
O. Yu. Olisova ◽  
E. Yu. Vertieva ◽  
D. R. Amshinskaya ◽  
M. G. Lecaillon ◽  
E. V. Grekova
Keyword(s):  
T Cell ◽  
Mycosis Fungoides ◽  
Cell Lymphoma ◽  
Correct Diagnosis ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
Not Otherwise Specified ◽  
T Cell Lymphomas ◽  
Diagnostic Difficulties ◽  
Life Threatening

Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) have an aggressive, life-threatening course. 5‑year survival rate is less than 20 %, which may be due to not timely diagnosis. PTCL-NOS can histologically and immunophenotypically mimic other T-cell lymphomas of the skin, including mycosis fungoides. In this connection, the correct diagnosis is most often established in the late stages of the disease. We present a clinical case of PTCL-NOS misdiagnosed as mycosis fungoides.

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

2017 ◽  
Vol 10 (1) ◽  
pp. 328-332 ◽  
Author(s):  
Kayo Tanita ◽  
Taku Fujimura ◽  
Yota Sato ◽  
Takanori Hidaka ◽  
Sadanori Furudate ◽  
...  
Keyword(s):  
T Cell ◽  
Mycosis Fungoides ◽  
Successful Treatment ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Cutaneous T Cell Lymphoma ◽  
Hematological Disease ◽  
Lymphoma Cells ◽  
Peripheral T Cell Lymphoma ◽  
Not Otherwise Specified

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.

ALK− anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project

Blood ◽  
2008 ◽  
Vol 111 (12) ◽  
pp. 5496-5504 ◽  
Author(s):  
Kerry J. Savage ◽  
Nancy Lee Harris ◽  
Julie M. Vose ◽  
Fred Ullrich ◽  
Elaine S. Jaffe ◽  
...  
Keyword(s):  
T Cell ◽  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
Anaplastic Lymphoma ◽  
Not Otherwise Specified ◽  
T Cell Lymphomas ◽  
Large Cell Lymphoma

Abstract The International Peripheral T-Cell Lymphoma Project is a collaborative effort designed to gain better understanding of peripheral T-cell and natural killer (NK)/T-cell lymphomas (PTCLs). A total of 22 institutions in North America, Europe, and Asia submitted clinical and pathologic information on PTCLs diagnosed and treated at their respective centers. Of the 1314 eligible patients, 181 had anaplastic large-cell lymphoma (ALCL; 13.8%) on consensus review: One hundred fifty-nine had systemic ALCL (12.1%) and 22 had primary cutaneous ALCL (1.7%). Patients with anaplastic lymphoma kinase–positive (ALK+) ALCL had a superior outcome compared with those with ALK− ALCL (5-year failure-free survival [FFS], 60% vs 36%; P = .015; 5-year overall survival [OS], 70% vs 49%; P = .016). However, contrary to prior reports, the 5-year FFS (36% vs 20%; P = .012) and OS (49% vs 32%; P = .032) were superior for ALK− ALCL compared with PTCL, not otherwise specified (PTCL-NOS). Patients with primary cutaneous ALCL had a very favorable 5-year OS (90%), but with a propensity to relapse (5-year FFS, 55%). In summary, ALK− ALCL should continue to be separated from both ALK+ ALCL and PTCL-NOS. Although the prognosis of ALK− ALCL appears to be better than that for PTCL-NOS, it is still unsatisfactory and better therapies are needed. Primary cutaneous ALCL is associated with an indolent course.

scholarly journals Potentials of brentuximab vedotin in the treatment of relapse/refractory cutaneous T-cell lymphomas: literature review and authors’ observation

2019 ◽  
Vol 95 (2) ◽  
pp. 42-49
Author(s):  
L. G. Gorenkova ◽  
S. K. Kravchenko ◽  
I. E. Belousova
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Brentuximab Vedotin ◽  
T Cell Lymphoma ◽  
Lymphoproliferative Diseases ◽  
Peripheral T Cell Lymphoma ◽  
Lymphomatoid Papulosis ◽  
Not Otherwise Specified ◽  
T Cell Lymphomas ◽  
Cutaneous Lymphomas

Primary cutaneous T-cell lymphomas encompass a heterogeneous group of T-cell lymphoproliferative disorders developing primarily in the skin and characterized by a number of specific diagnostic, clinical, and therapeutic features. Mycosis fungoides accounts for more than half of all cutaneous lymphoma cases, while CD30+ lymphoproliferative diseases of the skin (primary cutaneous anaplastic large-cell lymphoma and lymphomatoid papulosis) constitute one-fourth of them and the remaining cases are rare tumour types, including primary cutaneous peripheral T-cell lymphoma, unspecified/not otherwise specified.Activation antigen СD30 is a cell membrane glycoprotein of the tumour necrosis factor family. More than 75 % of primary cutaneous CD30-positive lymphoma cells express CD30; it may be detected in other diseases as well, but to a lesser extent.Most patients with cutaneous CD30+ lymphoproliferative diseases have indolent disease and a favourable prognosis; resistant disease is observed in approximately 30 % of sufferers, and fatal outcomes occur in 8 % of cases [1].Systemic immunomodulatory therapy or chemotherapy is often used in advanced disease. Monoclonal antibodies were recently introduced into clinical practice for the treatment of cutaneous lymphomas. One of these agents is brentuximab vedotin, a CD30-monoclonal antibody conjugated to monomethyl auristatin E.We present two case reports: one of frequently recurring lymphomatoid papulosis and the other of refractory primary cutaneous peripheral T-cell lymphoma, unspecified/not otherwise specified. Targeted therapy with brentuximab vedotin, either alone or in combination with chemotherapy, resulted in a sustained, long-lasting remission in both cases.

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.

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