Update on peripheral T-cell lymphomas with T-helper phenotype:  Are there too many subtypes?

Piris, Miguel Á.; Rodríguez‐Pinilla, Socorro María; Santonja, Carlos; Betancor, Isabel; Alonso‐Alonso, Ruth; Gru, Alejandro A.; Rodríguez, Marta

doi:10.1053/j.semdp.2019.12.005

Cited by 10 publications

(13 citation statements)

References 47 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…EBV‐positive activated B cells or Hodgkin/Reed–Sternberg (HRS)‐like cells are often observed in nTFHL‐AI and nTFHL‐F 3 . However, to the best of our knowledge this is the first report of EBV localised to the neoplastic TFH cells and background non‐neoplastic T cells.…”

Section: Discussionmentioning

confidence: 82%

A nodal EBV‐positive T cell lymphoma with a T follicular helper cell phenotype

Zhang

Wang

et al. 2023

Histopathology

View full text Add to dashboard Cite

Aims: Nodal T follicular helper (TFH) cell lymphoma (nTFHL) is a rare type of clinically aggressive T cell lymphoma. With this lymphoma type, Epstein-Barr virus (EBV) infection is frequently detected in nonneoplastic B lymphocytes, but not yet identified in neoplastic T cells. We report two cases of nTFHL showing a classic morphology and immunoprofile, with EBV-encoded small RNAs (EBER) in-situ hybridisation positivity in neoplastic TFH cells. Methods and results: Clonal T cell receptor (TR) gene rearrangement was detected in both cases. Whole exome sequencing identified TET2, RHOA p. G17V, as well as gene mutations unique to each case. Microdissection analysis showed EBER positivity in tumour cells and in background non-neoplastic T lymphocytes. Conclusion: These two immunocompetent cases of nTFHL with EBV-positive tumour cells exhibit the featured gene mutation profile and poor prognosis of this disease. This novel finding of EBV positivity in our cases expands the currently recognised spectrum of EBV-positive nodal T cell lymphomas to include rare cases of nTFHL.

show abstract

Section: Discussionmentioning

confidence: 82%

A nodal EBV‐positive T cell lymphoma with a T follicular helper cell phenotype

Zhang

Wang

et al. 2023

Histopathology

View full text Add to dashboard Cite

show abstract

“…Whilst this raises a theoretical question as to whether this case represents primary cutaneous TFH lymphoma, the data are insufficient for meaningful conclusions. In any event, a genuine TFH phenotype seems to be rare in primary cutaneous PTL NOS 29 …”

Section: Discussionmentioning

confidence: 95%

Primary cutaneous peripheral T‐cell lymphoma, not otherwise specified: results of a multicentre European Organization for Research and Treatment of Cancer (EORTC) cutaneous lymphoma taskforce study on the clinico‐pathological and prognostic features

Kempf

Mitteldorf

Battistella

et al. 2020

Acad Dermatol Venereol

View full text Add to dashboard Cite

Background Cutaneous peripheral T‐cell lymphoma, not otherwise specified (PTL NOS) is an aggressive, but poorly characterized neoplasm. Objectives The European Organization for Research and Treatment of Cancer cutaneous lymphoma taskforce (EORTC CLTF) investigated 33 biopsies of 30 patients with primary cutaneous PTL NOS to analyse their clinical, histological, immunophenotypic features and outcome. Methods Retrospective analysis of clinical data and histopathological features by an expert panel. Results Cutaneous PTL NOS manifested clinically either with solitary or disseminated rapidly grown ulcerated tumours or disseminated papulo‐nodular lesions. Histologically, a mostly diffuse or nodular infiltrate in the dermis and often extending into the subcutis was found. Epidermotropism was rarely present and only mild and focal. Unusual phenotypes were frequent, e.g. CD3+/CD4−/CD8− and CD3+/CD4+/CD8+. Moreover, 18% of the cases exhibited an aberrant expression of the B‐cell marker CD20 by the tumour cells. All solitary tumours were located on the limbs and presented a high expression of GATA‐3 but this did not correlate with outcome and therefore could not serve as a prognostic factor. The prognosis was shown to be generally poor with 10 of 30 patients (33%) dying of lymphoma within the follow‐up of 36 months (mean value; range 3–144). The survival rates were 61% after 3 years (CI, 43–85%) and 54% after 5 years (CI, 36–81%). Small to medium‐sized morphology of tumour cells was associated with a better outcome than medium to large or large tumour cells. Age, gender, clinical stage, CD4/CD8 phenotype and GATA‐3 expression were not associated with prognosis. Chemotherapy was the most common treatment modality, but surgical excision and/or radiotherapy may represent an appropriate first‐line treatment for solitary lesions. Conclusions Cutaneous PTL NOS shows an aggressive course in most patients independent of initial presentation, age and phenotype. Cytomorphology was identified as a prognostic factor. The data indicate a need for more effective treatment modalities in PTL NOS.

show abstract

“…The CD8+ antigen, a T lymphocyte surface marker, can reflect cellular immune response. In an enhanced humoral immune response, the CD4 + cells count increases, and CD8 + cell count decreases, whereas the CD4 + to CD8 + ratio increases (Piris et al., 2020 ). Thus, the CD4 + to CD8 + ratio regulates the activation and inhibition of the immune system (Arroyo & Pello, 2020 ; He et al., 2020 ).…”

Section: Resultsmentioning

confidence: 99%