T- and NK-cell lymphoproliferative disorders of the gastrointestinal tract: review and update

Pathology. 2020 Jan;52(1):128-141. doi: 10.1016/j.pathol.2019.10.001. Epub 2019 Nov 11.


T- and NK-cell lymphoproliferative disorders of the gastrointestinal (GI) tract are uncommon, but are important to recognise as there may be morphological and immunophenotypic overlap between lymphoid lesions with vastly different clinical outcomes. Recent data have led to the reclassification of some lymphomas and inclusion of new entities in the 2016 revision of World Health Organization (WHO) classification of lymphoid neoplasms. It has become clear that enteropathy associated T-cell lymphoma (EATL), formerly thought to be composed of two subtypes known as type I and type II, are distinct entities. Type I EATL is now simply classified as EATL; it is strongly associated with coeliac disease and occurs mainly in Western populations. Type II EATL has been renamed monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL); it shows no definite association with coeliac disease and occurs worldwide with a predominance in Asian populations. There is also a group of aggressive intestinal T-cell lymphomas which do not meet criteria for EATL, MEITL, extranodal NK/T-cell lymphoma of nasal type or anaplastic large cell lymphoma. These neoplasms are now designated intestinal T-cell lymphoma, not otherwise specified. Indolent T-cell lymphoproliferative disorder of the GI tract has been included as a provisional entity in the most recent WHO classification. It is a clonal T-cell lymphoproliferative disorder (CD4+ or CD8+) with an indolent clinical course. Finally, benign NK-cell proliferations of the GI tract, variably designated 'NK-cell enteropathy' and 'lymphomatoid gastropathy' have also been recognised in the last two decades but have not been included in the WHO classification as their neoplastic nature is not established. This review covers the aforementioned lymphoid proliferations, emphasising their salient clinicopathological features and genetic abnormalities. It also provides practical insights into resolving difficult differential diagnoses in daily surgical pathology practice.

Keywords: Intestinal T-cell lymphoma; NK-cell enteropathy; benign NK-cell proliferation of the gastrointestinal tract; cavitating mesenteric lymph node syndrome; enteropathy-associated T-cell lymphoma; indolent T-cell lymphoproliferative disorder of the gastrointestinal tract; intestinal T-cell lymphoma not otherwise specified; lymphomatoid gastropathy; monomorphic epitheliotropic intestinal T-cell lymphoma; refractory coeliac disease.

Publication types

  • Review

MeSH terms

  • Gastrointestinal Diseases / immunology
  • Gastrointestinal Diseases / pathology
  • Gastrointestinal Tract / pathology*
  • Humans
  • Killer Cells, Natural / immunology
  • Killer Cells, Natural / pathology*
  • Lymphoma, Extranodal NK-T-Cell / immunology
  • Lymphoma, Extranodal NK-T-Cell / pathology*
  • Lymphoproliferative Disorders / immunology
  • Lymphoproliferative Disorders / pathology*
  • T-Lymphocytes / immunology
  • T-Lymphocytes / pathology