Duodenal-type follicular lymphoma (DFL) is a rare variant of follicular lymphoma (FL) characterized by distinctive clinical features such as localization and favorable prognosis. We herein report a case of DFL in which histological transformation into diffuse large B-cell lymphoma developed 7 years after diagnosis. The transformed lymphoma was refractory to chemotherapy, and the patient passed away due to disease progression. To date, there have been only a limited number of reported cases of histological transformation of DFL, and the clinical outcomes of those cases except our present case have been favorable, with good responses to chemotherapy. Although the histological transformation of DFL is a rare event, the clinical course of the present case suggested that it would be a fatal event and underscore the importance of the life-long management of DFL. Further accumulation of cases is required to elucidate its incidence, characteristics, and prognosis.
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