scholarly journals An overview of cutaneous T cell lymphomas

F1000Research ◽  
2016 ◽  
Vol 5 ◽  
pp. 1882 ◽  
Author(s):  
Nooshin Bagherani ◽  
Bruce R. Smoller
Keyword(s):  
T Cell ◽  
T Lymphocytes ◽  
Mycosis Fungoides ◽  
Annual Incidence ◽  
Heterogeneous Group ◽  
Not Otherwise Specified ◽  
T Cell Lymphomas ◽  
Hodgkin's Lymphomas ◽  
Diagnosis Therapy ◽  
Peripheral T Cell Lymphomas

Cutaneous T cell lymphomas (CTCLs) are a heterogeneous group of extranodal non-Hodgkin’s lymphomas that are characterized by a cutaneous infiltration of malignant monoclonal T lymphocytes. They typically afflict adults with a median age of 55 to 60 years, and the annual incidence is about 0.5 per 100,000. Mycosis fungoides, Sézary syndrome, and primary cutaneous peripheral T cell lymphomas not otherwise specified are the most important subtypes of CTCL. CTCL is a complicated concept in terms of etiopathogenesis, diagnosis, therapy, and prognosis. Herein, we summarize advances which have been achieved in these fields.

scholarly journals Cutaneous T-cell lymphomas: modern data of pathogenesis, clinics and therapy

2018 ◽  
Vol 13 (3) ◽  
pp. 25-38
Author(s):  
O. M. Demina ◽  
O. E. Akilov ◽  
A. G. Rumyantsev
Keyword(s):  
T Cell ◽  
T Lymphocytes ◽  
Mycosis Fungoides ◽  
Lymphoproliferative Diseases ◽  
Complex Concept ◽  
T Cell Lymphomas ◽  
Skin Infiltration ◽  
Hodgkin's Lymphomas ◽  
Diagnosis Therapy ◽  
Summary Data

Cutaneous T-cell lymphomas (CTCL) are a heterogeneous group of extranodal non-Hodgkin’s lymphomas that are characterized by skin infiltration with malignant monoclonal T lymphocytes. More common in adults aged 55 to 60 years, the annual incidence is about 0.5 per 100 000 people. Mycosis fungoides, Sézary syndrome and CD30+ lymphoproliferative diseases are the main subtypes of CTCL. To date, CTCL have a complex concept of etiopathogenesis, diagnosis, therapy and prognosis. The article presented summary data on these issues.

scholarly journals The expression of CD26 and CD40 ligand is mutually exclusive in human T- cell non-Hodgkin's lymphomas/leukemias

Blood ◽  
1995 ◽  
Vol 86 (12) ◽  
pp. 4617-4626 ◽  
Author(s):  
A Carbone ◽  
A Gloghini ◽  
V Zagonel ◽  
D Aldinucci ◽  
V Gattei ◽  
...  
Keyword(s):  
T Cells ◽  
T Cell ◽  
T Lymphocytes ◽  
Mycosis Fungoides ◽  
Cell Lines ◽  
Critical Role ◽  
Cd40 Ligand ◽  
T Cell Lymphomas ◽  
Human T Cell ◽  
Hodgkin's Lymphomas

CD26 and CD40 ligand (CD40L) are surface molecules on human activated T lymphocytes that play a critical role in the regulation of lymphopoiesis. Both molecules are expressed on a restricted fraction of human T-cell non-Hodgkin's lymphomas (NHL)/leukemias; however, little is known about their functional and/or clinical significance in these disorders. In this study, the pattern of expression of CD40L was compared with that of the CD26 molecule. A series of 67 human T-cell NHL/leukemias and a panel of leukemia/lymphoma T-cell lines were evaluated by immunohistochemistry, flow cytometry, and RNA studies. The overall frequency of CD26+ and CD40L+ samples was rather similar (25/67 [37%] v 18/67 [27%]). However, the majority of CD26-expressing cases clustered in the lymphoblastic lymphomas (LBL)/T-acute lymphoblastic leukemias (ALL; 12/23) and CD30+ anaplastic large-cell (ALC) lymphomas (5/8), whereas CD40L+ lymphomas included a large fraction of mycosis fungoides (11/21 [52%]). CD26 and CD40L coexpression was found only in 2 myocosis fungoides cases and 1 small lymphocytic lymphoma. Thus, the expression of the two antigens was mutually exclusive in almost all T- cell lymphomas/leukemias. Accordingly, lymphoma cell lines expressed either one of the molecules or the relative amounts of CD26 and CD40L were inversely proportional. In contrast, reactive T lymphocytes from patients with non-neoplastic T-cell expansions and in vitro activated CD3+ or CD4+ normal T cells were found to coexpress CD40L and CD26. Results of a multivariate analysis showed that the expression of CD26 in T-cell LBL/ALL patients was associated to a worse outcome in terms of survival, as compared with patients with CD26- tumors (P < or = .0001). Based on our results, it can be concluded that, (1) as opposed to activated or reactive normal T cells, the expression of CD26 and of CD40L is mutually exclusive in human T-cell lymphomas/leukemias; (2) expression of CD26 is restricted to aggressive pathologic entities, such as T-cell LBL/ALL and T-cell CD30+ ALC lymphomas, whereas CD40L is expressed on slow progressing diseases such as mycosis fungoides; and (3) within the T-cell LBL/ALL group of tumors, CD26 may identify a subset of poor prognosis patients.

Peripheral T-cell lymphomas, unspecified (or not otherwise specified): a review

2008 ◽  
Vol 26 (1) ◽  
pp. 8-20 ◽  
Author(s):  
Delvys Rodriguez-Abreu ◽  
Volmar Belisario Filho ◽  
Emanuele Zucca
Keyword(s):  
T Cell ◽  
Not Otherwise Specified ◽  
T Cell Lymphomas ◽  
Peripheral T Cell Lymphomas

scholarly journals Granzyme B-expressing peripheral T-cell lymphomas: neoplastic equivalents of activated cytotoxic T cells with preference for mucosa- associated lymphoid tissue localization

Blood ◽  
1994 ◽  
Vol 84 (11) ◽  
pp. 3785-3791 ◽  
Author(s):  
PC de Bruin ◽  
JA Kummer ◽  
P van der Valk ◽  
P van Heerde ◽  
PM Kluin ◽  
...  
Keyword(s):  
T Cells ◽  
T Cell ◽  
Tumor Cells ◽  
Lymphoid Tissue ◽  
Cytotoxic T Cells ◽  
Granzyme B ◽  
Clinical Behavior ◽  
T Cell Lymphomas ◽  
Hodgkin's Lymphomas ◽  
Peripheral T Cell Lymphomas

T-cell non-Hodgkin's lymphomas can be considered the neoplastic equivalents of immunologically functional, site-restricted T lymphocytes. Little is known about the occurrence and clinical behavior of T-cell lymphomas that are the neoplastic equivalents of different functional T-cell subsets. Here, we investigated the prevalence, preferential site, immunophenotype, and clinical behavior of the neoplastic equivalents of activated cytotoxic T cells (CTLs) in a group of 140 nodal and extranodal T-cell lymphomas. Activated CTLs were shown immunohistochemically with a monoclonal antibody against granzyme B, a major constituent of the cytotoxic granules of activated T cells. Granzyme B-positive T-cell lymphomas were mainly found in mucosa- associated lymphoid tissue (MALT; nose, 63% of the cases; gastrointestinal tract, 46%; and lung, 33%). Granzyme B-positive cases with primary localization in MALT were more often associated with angioinvasion (P = .005), necrosis (P = .002), and histologic characteristics of celiac disease in adjacent mucosa not involved with lymphoma. Eosinophilia was more often observed in granzyme B-negative cases (P = .03). Most cases belonged to the pleomorphic medium- and large-cell group of the Kiel classification. CD30 expression was more often found in granzyme B-positive lymphomas of MALT (P = .04), whereas CD56 expression was exclusively found in nasal granzyme B-positive lymphomas. Immunophenotypically, most of the cases should be considered as neoplastic equivalents of activated CTLs based on the presence of T- cell markers on tumor cells. In two cases of nasal lymphoma, tumor cells probably were the neoplastic counterparts of natural killer cells. The prognosis of the granzyme B-positive gastrointestinal T-cell lymphomas was poor but did not differ from granzyme B-negative gastrointestinal T-cell lymphomas. This indicates that, in peripheral T- cell lymphomas, site of origin is more important as a prognostic parameter than derivation of activated CTLs.

scholarly journals Recurrent TET2 mutations in peripheral T-cell lymphomas correlate with TFH-like features and adverse clinical parameters

Blood ◽  
2012 ◽  
Vol 120 (7) ◽  
pp. 1466-1469 ◽  
Author(s):  
François Lemonnier ◽  
Lucile Couronné ◽  
Marie Parrens ◽  
Jean-Philippe Jaïs ◽  
Marion Travert ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
International Prognostic Index ◽  
Prognostic Index ◽  
Progression Free Survival ◽  
T Cell Lymphoma ◽  
Not Otherwise Specified ◽  
T Cell Lymphomas ◽  
Stage Disease ◽  
Peripheral T Cell Lymphomas

Abstract Inactivating mutations of the Ten-Eleven Translocation 2 (TET2) gene were first identified in myeloid malignancies and more recently in peripheral T-cell lymphomas (PTCLs). In the present study, we investigated the presence of TET2 coding sequence mutations and their clinical relevance in a large cohort of 190 PTCL patients. TET2 mutations were identified in 40 of 86 (47%) cases of angioimmunoblastic T-cell lymphoma (AITL) and in 22 of 58 (38%) cases of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), but were absent in all other PTCL entities, with the exception of 2 of 10 cases of enteropathy-associated T-cell lymphoma. Among PTCL-NOS, a heterogeneous group of lymphoma-comprising cases likely to derive from Th follicular (TFH) cells similarly to AITL, TET2 mutations were more frequent when PTCL-NOS expressed TFH markers and/or had features reminiscent of AITL (58% vs 24%, P = .01). In the AITL and PTCL-NOS subgroups, TET2 mutations were associated with advanced-stage disease, thrombocytopenia, high International Prognostic Index scores, and a shorter progression-free survival.

scholarly journals Biology of peripheral T cell lymphomas – Not otherwise specified: Is something finally happening?

Pathogenesis ◽  
2016 ◽  
Vol 3 (1) ◽  
pp. 9-18 ◽  
Author(s):  
Francesco Maura ◽  
Anna Dodero ◽  
Cristiana Carniti ◽  
Niccolò Bolli
Keyword(s):  
T Cell ◽  
Not Otherwise Specified ◽  
T Cell Lymphomas ◽  
Peripheral T Cell Lymphomas

scholarly journals Pralatrexate in patients with recurrent or refractory peripheral T-cell lymphomas: a multicenter retrospective analysis

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Jung Yong Hong ◽  
Dok Hyun Yoon ◽  
Sang Eun Yoon ◽  
Seok Jin Kim ◽  
Ho Sup Lee ◽  
...  
Keyword(s):  
T Cell ◽  
Retrospective Analysis ◽  
Real World ◽  
Dose Intensity ◽  
Progression Free Survival ◽  
Complete Response ◽  
Efficacy And Safety ◽  
T Cell Lymphomas ◽  
Hodgkin's Lymphomas ◽  
Peripheral T Cell Lymphomas

AbstractPeripheral T-cell lymphomas (PTCL) are a heterogeneous group of non-Hodgkin’s lymphomas with poor clinical outcomes. Pralatrexate showed efficacy and safety in recurrent or refractory PTCLs. The purpose or this study was to investigate the efficacy and safety of pralatrexate in relapsed or refractory PTCLs in real-world practice. This was an observational, multicenter, retrospective analysis. Between December 2012 and December 2016, a total of 38 patients with relapsed or refractory PTCLs were treated with pralatrexate at 10 tertiary hospitals in Korea. Patients received an intravenous infusion of pralatrexate at a dose of 30 mg/m2/week for 6 weeks on a 7-week schedule. Modified dosing and/or scheduling was allowed according to institutional protocols. Median patient age was 58 years (range, 29–80 years) and the most common subtype was peripheral T-cell lymphoma, not otherwise specified (n = 23, 60.5%). The median dosage of pralatrexate per administration was 25.6 mg/m2/wk (range, 15.0–33.0 mg/m2/wk). In intention-to-treat analysis, 3 patients (7.9%) showed a complete response and 5 patients (13.2%) showed a partial response, resulting in an overall response rate (ORR) of 21.1%. The median duration of response was 7.6 months (range, 1.6–24.3 months). The median progression-free survival (PFS) was 1.8 months (95% confidence interval [CI], 1.7–1.8 months) and the median overall survival was 7.7 months (95% CI, 4.4–9.0 months). The most common grade 3/4 adverse events were thrombocytopenia (n = 13, 34.2%), neutropenia (n = 7, 23.7%), and anemia (n = 7, 18.4%). Our study showed relatively lower ORR and shorter PFS in patients with recurrent or refractory PTCLs treated with pralatrexate in real-world practice. The toxicity profile was acceptable and manageable. We also observed significantly lower dose intensity of pralatrexate in real-world practice.

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.

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