An Isolated Mesenteric Presentation of a Nodal Peripheral T Cell Lymphoma with T Follicular Helper Cell Phenotype

Anna Keogh; Fiona Lynott; Antonios Papanicolau-Sengos; Mutaz Mohammed Nur; Aisling Spillane; Fiona Quinn; Ezzat ElHassadi; Elaine S Jaffe; Richard Flavin

doi:10.3390/hematolrep14040047

An Isolated Mesenteric Presentation of a Nodal Peripheral T Cell Lymphoma with T Follicular Helper Cell Phenotype

Hematol Rep. 2022 Nov 15;14(4):335-341. doi: 10.3390/hematolrep14040047.

Authors

Anna Keogh^{1

2}, Fiona Lynott³, Antonios Papanicolau-Sengos³, Mutaz Mohammed Nur⁴, Aisling Spillane⁵, Fiona Quinn⁵, Ezzat ElHassadi⁶, Elaine S Jaffe⁶, Richard Flavin^{1

2}

Affiliations

¹ Department of Histopathology, Saint James Hospital, D08 NHY1 Dublin, Ireland.
² Department of Histopathology, Trinity College Dublin, D02 PN40 Dublin, Ireland.
³ Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20814, USA.
⁴ Department of Histopathology, University Hospital Waterford, X91 ER8E Waterford, Ireland.
⁵ Cancer Molecular Diagnostics Department, Saint James Hospital, D08 NHY1 Dublin, Ireland.
⁶ Department of Haematology, University Hospital Waterford, X91 ER8E Waterford, Ireland.

Abstract

Nodal peripheral T cell lymphoma (PTCL) with T follicular helper (TFH) cell phenotype is a provisional entity added to the 2016 revised WHO classification of haematological malignancies. These lymphomas have an aggressive clinical course and respond poorly to conventional treatments. Distinct histological features have not been well described. Additionally, the minimum criteria for diagnosis is not well established but detection of at least two TFH markers in addition to CD4 is suggested to assign a TFH cell phenotype. Some pathological features of angioimmunoblastic T cell lymphoma (AITL) such as recurrent molecular alterations are commonly found. As the name suggests, these lymphomas are nodal in origin with patients presenting with widespread lymphadenopathy. We describe the first documented case of nodal PTCL with a TFH phenotype presenting as an isolated mesenteric mass with no nodal involvement.

Keywords: T follicular helper cell origin 4; extra-nodal 2; mesenteric mass 3; nodal PTCL with TFH phenotype 1.

Publication types

Case Reports

Grants and funding

This work was supported by the intramural research program of the Center for Cancer Research, National Cancer Institute, National Institutes of Health.