Indolent T Cell Lymphoproliferation of the Gastrointestinal Tract: An Evolving Disease Entity

Autor: Luke Wang, Elaine Koh, Beena Kumar, Michael S. Y. Low
Jazyk: angličtina
Rok vydání: 2024
Předmět:
Zdroj: Hematology Reports, Vol 16, Iss 2, Pp 179-184 (2024)
Druh dokumentu: article
ISSN: 2038-8330
46801510
DOI: 10.3390/hematolrep16020018
Popis: Background: Indolent T cell lymphoproliferation of the gastrointestinal tract is a novel entity recently added to the 2016 WHO classification of lymphoid neoplasms. Classically, these patients demonstrate an immunophenotype consistent with T cell proliferation and can be either CD4-positive or CD8-positive but with a low Ki67 index, highlighting the indolent nature of this disease compared to its more aggressive T cell lymphoma counterparts such as enteropathy-associated T cell lymphoma and monomorphic epitheliotropic intestinal T cell lymphoma. Methods: Here, we describe one rare case of such a neoplasm under our care, initially presenting with non-specific signs and symptoms and requiring extensive investigations to diagnose. Available cases in the literature reflect a wide variety of ages and ethnicities affected, and any part of the gastrointestinal sites can be affected, which makes diagnosis difficult and prolonged; however, progression beyond lymph nodes is rare, and prognosis is otherwise favourable, particularly if CD8-positive. The optimal management of these patients remains yet to be defined, given the paucity of available cases currently. The current evidence suggests the utility of steroids, cyclosporine, radiotherapy, and a potential role for JAK inhibitors. Conclusions: Our case showed an excellent response to the initial course of steroids, with a subsequent successful transition to cyclosporine, keeping symptoms at bay with ongoing stable disease.
Databáze: Directory of Open Access Journals
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