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Abstract Introduction Primary intestinal T-cell lymphomas are rare diseases (5% of GI tract lymphomas). Thus, a diagnostic approach, including clinical, morphologic, and immunophenotypic features, usually leads to a preliminary diagnosis of mature T-cell lymphoma. The currently available data on clinico-pathological aspects of primary intestinal T-cell lymphomas are very limited in developing countries. This study highlights the challenges in the diagnosis of this rare disease in such an exceptional presentation. Case description This is a retrospective analysis on clinico pathological characteristics of six primary intestinal T-cell lymphoma’s cases diagnosed upon surgical specimen in the pathology department (period of 8 years). There were four females and two males with a median age of 54 years. The two histologic subtypes of primary intestinal T-cell lymphomas were enteropathy-associated T-cell lymphoma (five cases) and intestinal T-cell lymphoma NOS (one case). Discussion In conclusion, this limited case series of this disease from Morocco illustrates the scarcity of these neoplasms, emphasizes challenges in their diagnosis, and reflects their high mortality. Learning points Highlight the scarcity of these neoplasms and the diagnostic challenges. Expand knowledge about these tumors. Provide a literature review of these lymphoma.
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DOI: 10.1093/rescon/vmaf003
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