Cutaneous lesions of angioimmunoblastic T-cell lymphoma: Clinical, pathological, and immunophenotypic features

Naoki Oishi, Julio Sartori Valinotti, Nabila Bennani, David A. Wada, Rong He, Mark A. Cappel, Andrew L Feldman

Research output: Contribution to journalArticle

Abstract

Background: Angioimmunoblastic T-cell lymphoma (AITL) is a systemic peripheral T-cell lymphoma with a follicular helper T-cell (T FH ) immunophenotype that frequently involves the skin. However, the histopathology of cutaneous involvement by AITL has not been fully established. Methods: We reviewed the clinicopathological features of 19 patients seen at our institution with AITL involving the skin. Pan-T-cell and T FH marker expression was evaluated by immunohistochemistry. Epstein-Barr virus (EBV) was detected using in situ hybridization (ISH) for Epstein-Barr virus-encoded small RNA (EBER). T-cell receptor (TCR) gene rearrangement was evaluated by PCR. Results: AITL affected both trunk and extremities in 15/19 cases (79%). Perivascular infiltration by small and/or medium-sized lymphocytes was seen in 18/19 (95%). Granulomatous inflammation was identified in 4/19 (21%). Aberrant loss of CD2, CD5, or CD7 was identified in 1/18 (6%), 2/18 (11%), or 7/19 (37%) cases, respectively. Seventeen of eighteen evaluable cases (95%) expressed 2 to 3 T FH markers: PD-1 in 19/19 (100%), BCL6 in 94% (17/18), and CD10 in 37% (7/19). EBV-positive cells were detected in 3/18 (17%) with varying density. Clonal TCR gene rearrangement was identified in 9/11 (82%). Conclusions: Cutaneous involvement by AITL shows relatively non-specific histopathological features. However, an immunohistochemical panel including T FH markers and EBER ISH is useful in differential diagnosis.

Original languageEnglish (US)
JournalJournal of Cutaneous Pathology
DOIs
StatePublished - Jan 1 2019

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T-Cell Lymphoma
Human Herpesvirus 4
Skin
T-Lymphocyte Gene Rearrangement
T-Cell Receptor Genes
In Situ Hybridization
RNA
Peripheral T-Cell Lymphoma
Helper-Inducer T-Lymphocytes
Differential Diagnosis
Extremities
Immunohistochemistry
Lymphocytes
Inflammation
T-Lymphocytes
Polymerase Chain Reaction

Keywords

  • angioimmunoblastic T-cell lymphoma
  • angioimmunoblastic T-cell lymphoma
  • cutaneous lymphoma
  • cutaneous lymphoma
  • Epstein-Barr virus
  • Epstein-Barr virus
  • follicular helper T-cell
  • follicular helper T-cell

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Dermatology

Cite this

@article{bf4347445dda4dd0b5468edbabb801ef,
title = "Cutaneous lesions of angioimmunoblastic T-cell lymphoma: Clinical, pathological, and immunophenotypic features",
abstract = "Background: Angioimmunoblastic T-cell lymphoma (AITL) is a systemic peripheral T-cell lymphoma with a follicular helper T-cell (T FH ) immunophenotype that frequently involves the skin. However, the histopathology of cutaneous involvement by AITL has not been fully established. Methods: We reviewed the clinicopathological features of 19 patients seen at our institution with AITL involving the skin. Pan-T-cell and T FH marker expression was evaluated by immunohistochemistry. Epstein-Barr virus (EBV) was detected using in situ hybridization (ISH) for Epstein-Barr virus-encoded small RNA (EBER). T-cell receptor (TCR) gene rearrangement was evaluated by PCR. Results: AITL affected both trunk and extremities in 15/19 cases (79{\%}). Perivascular infiltration by small and/or medium-sized lymphocytes was seen in 18/19 (95{\%}). Granulomatous inflammation was identified in 4/19 (21{\%}). Aberrant loss of CD2, CD5, or CD7 was identified in 1/18 (6{\%}), 2/18 (11{\%}), or 7/19 (37{\%}) cases, respectively. Seventeen of eighteen evaluable cases (95{\%}) expressed 2 to 3 T FH markers: PD-1 in 19/19 (100{\%}), BCL6 in 94{\%} (17/18), and CD10 in 37{\%} (7/19). EBV-positive cells were detected in 3/18 (17{\%}) with varying density. Clonal TCR gene rearrangement was identified in 9/11 (82{\%}). Conclusions: Cutaneous involvement by AITL shows relatively non-specific histopathological features. However, an immunohistochemical panel including T FH markers and EBER ISH is useful in differential diagnosis.",
keywords = "angioimmunoblastic T-cell lymphoma, angioimmunoblastic T-cell lymphoma, cutaneous lymphoma, cutaneous lymphoma, Epstein-Barr virus, Epstein-Barr virus, follicular helper T-cell, follicular helper T-cell",
author = "Naoki Oishi and {Sartori Valinotti}, Julio and Nabila Bennani and Wada, {David A.} and Rong He and Cappel, {Mark A.} and Feldman, {Andrew L}",
year = "2019",
month = "1",
day = "1",
doi = "10.1111/cup.13475",
language = "English (US)",
journal = "Journal of Cutaneous Pathology",
issn = "0303-6987",
publisher = "Wiley-Blackwell",

}

TY - JOUR

T1 - Cutaneous lesions of angioimmunoblastic T-cell lymphoma

T2 - Clinical, pathological, and immunophenotypic features

AU - Oishi, Naoki

AU - Sartori Valinotti, Julio

AU - Bennani, Nabila

AU - Wada, David A.

AU - He, Rong

AU - Cappel, Mark A.

AU - Feldman, Andrew L

PY - 2019/1/1

Y1 - 2019/1/1

N2 - Background: Angioimmunoblastic T-cell lymphoma (AITL) is a systemic peripheral T-cell lymphoma with a follicular helper T-cell (T FH ) immunophenotype that frequently involves the skin. However, the histopathology of cutaneous involvement by AITL has not been fully established. Methods: We reviewed the clinicopathological features of 19 patients seen at our institution with AITL involving the skin. Pan-T-cell and T FH marker expression was evaluated by immunohistochemistry. Epstein-Barr virus (EBV) was detected using in situ hybridization (ISH) for Epstein-Barr virus-encoded small RNA (EBER). T-cell receptor (TCR) gene rearrangement was evaluated by PCR. Results: AITL affected both trunk and extremities in 15/19 cases (79%). Perivascular infiltration by small and/or medium-sized lymphocytes was seen in 18/19 (95%). Granulomatous inflammation was identified in 4/19 (21%). Aberrant loss of CD2, CD5, or CD7 was identified in 1/18 (6%), 2/18 (11%), or 7/19 (37%) cases, respectively. Seventeen of eighteen evaluable cases (95%) expressed 2 to 3 T FH markers: PD-1 in 19/19 (100%), BCL6 in 94% (17/18), and CD10 in 37% (7/19). EBV-positive cells were detected in 3/18 (17%) with varying density. Clonal TCR gene rearrangement was identified in 9/11 (82%). Conclusions: Cutaneous involvement by AITL shows relatively non-specific histopathological features. However, an immunohistochemical panel including T FH markers and EBER ISH is useful in differential diagnosis.

AB - Background: Angioimmunoblastic T-cell lymphoma (AITL) is a systemic peripheral T-cell lymphoma with a follicular helper T-cell (T FH ) immunophenotype that frequently involves the skin. However, the histopathology of cutaneous involvement by AITL has not been fully established. Methods: We reviewed the clinicopathological features of 19 patients seen at our institution with AITL involving the skin. Pan-T-cell and T FH marker expression was evaluated by immunohistochemistry. Epstein-Barr virus (EBV) was detected using in situ hybridization (ISH) for Epstein-Barr virus-encoded small RNA (EBER). T-cell receptor (TCR) gene rearrangement was evaluated by PCR. Results: AITL affected both trunk and extremities in 15/19 cases (79%). Perivascular infiltration by small and/or medium-sized lymphocytes was seen in 18/19 (95%). Granulomatous inflammation was identified in 4/19 (21%). Aberrant loss of CD2, CD5, or CD7 was identified in 1/18 (6%), 2/18 (11%), or 7/19 (37%) cases, respectively. Seventeen of eighteen evaluable cases (95%) expressed 2 to 3 T FH markers: PD-1 in 19/19 (100%), BCL6 in 94% (17/18), and CD10 in 37% (7/19). EBV-positive cells were detected in 3/18 (17%) with varying density. Clonal TCR gene rearrangement was identified in 9/11 (82%). Conclusions: Cutaneous involvement by AITL shows relatively non-specific histopathological features. However, an immunohistochemical panel including T FH markers and EBER ISH is useful in differential diagnosis.

KW - angioimmunoblastic T-cell lymphoma

KW - angioimmunoblastic T-cell lymphoma

KW - cutaneous lymphoma

KW - cutaneous lymphoma

KW - Epstein-Barr virus

KW - Epstein-Barr virus

KW - follicular helper T-cell

KW - follicular helper T-cell

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U2 - 10.1111/cup.13475

DO - 10.1111/cup.13475

M3 - Article

C2 - 30980412

AN - SCOPUS:85065226924

JO - Journal of Cutaneous Pathology

JF - Journal of Cutaneous Pathology

SN - 0303-6987

ER -