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  1. Enteropathy-associated T-cell Lymphoma (EATL) with intracranial metastasis : a rare and dismal condition
    Chuah YY, Tashi T, Lee YY, Fu TY, Shih CA
    Acta Gastroenterol Belg, 2020 4 2;83(1):77-80.
    PMID: 32233276
    Background: Enteropathy-associated T-cell lymphoma (EATL) is a rare type of gastrointestinal non-Hodgkin's Lymphoma. EATL with intracranial metastasis is even rarer. We report a case of EATL with intracranial metastasis.

    Case Presentation: A 36-years old man presented with five weeks history of intractable diarrhea. Colonoscopy was normal, but abdominal computed tomography (CT) scan revealed mural thickening at duodenojejunal junction, and subsequent jejunofiberoscopy showed a circumferential ulceration at the jejunum. Histo-immunopathology confirmed the diagnosis of enteropathyassociated T-cell lymphoma (EATL) type II. His disease course proved to be aggressive and refractory to standard front-line chemotherapy, and eventually progressed through second-line salvage regimen with CNS and intracranial involvement. He died nine months after the initial diagnosis.

    Conclusion: EATL with brain metastasis is a very rare occurrence with dismal prognosis.

    Matched MeSH terms: Enteropathy-Associated T-Cell Lymphoma*
  2. Fulltext Enteropathy-associated T-cell lymphoma: An extremely rare cause of chronic diarrhoea
    Liong CC, Ravindran S, Gnana Kumar G, Chin EF, Koh PS, Chan WK
    Med J Malaysia, 2016 04;71(2):88-90.
    PMID: 27326953 MyJurnal
    Chronic diarrhoea in tropical countries may be due to a myriad of causes from infective to non-infective. This case report illustrates the challenges faced in the investigation of a middle-age Chinese gentleman who presented with chronic diarrhoea and weight loss. The diagnosis of type II enteropathy-associated T-cell lymphoma (EATL) was finally made. The diagnosis of EATL was least suspected as the condition is almost unheard of in this part of the world. The epidemiology, presentation, diagnosis, management and prognosis of this rare condition are discussed.
    Matched MeSH terms: Enteropathy-Associated T-Cell Lymphoma/diagnosis*
  3. Fulltext JAK-STAT and G-protein-coupled receptor signaling pathways are frequently altered in epitheliotropic intestinal T-cell lymphoma
    Nairismägi ML, Tan J, Lim JQ, Nagarajan S, Ng CC, Rajasegaran V, et al.
    Leukemia, 2016 06;30(6):1311-9.
    PMID: 26854024 DOI: 10.1038/leu.2016.13
    Epitheliotropic intestinal T-cell lymphoma (EITL, also known as type II enteropathy-associated T-cell lymphoma) is an aggressive intestinal disease with poor prognosis and its molecular alterations have not been comprehensively characterized. We aimed to identify actionable easy-to-screen alterations that would allow better diagnostics and/or treatment of this deadly disease. By performing whole-exome sequencing of four EITL tumor-normal pairs, followed by amplicon deep sequencing of 42 tumor samples, frequent alterations of the JAK-STAT and G-protein-coupled receptor (GPCR) signaling pathways were discovered in a large portion of samples. Specifically, STAT5B was mutated in a remarkable 63% of cases, JAK3 in 35% and GNAI2 in 24%, with the majority occurring at known activating hotspots in key functional domains. Moreover, STAT5B locus carried copy-neutral loss of heterozygosity resulting in the duplication of the mutant copy, suggesting the importance of mutant STAT5B dosage for the development of EITL. Dysregulation of the JAK-STAT and GPCR pathways was also supported by gene expression profiling and further verified in patient tumor samples. In vitro overexpression of GNAI2 mutants led to the upregulation of pERK1/2, a member of MEK-ERK pathway. Notably, inhibitors of both JAK-STAT and MEK-ERK pathways effectively reduced viability of patient-derived primary EITL cells, indicating potential therapeutic strategies for this neoplasm with no effective treatment currently available.
    Matched MeSH terms: Enteropathy-Associated T-Cell Lymphoma/metabolism*; Enteropathy-Associated T-Cell Lymphoma/pathology
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