The simultaneous presence of hematological malignancies and sarcoidosis, defined as sarcoidosis-lymphoma syndrome, has been reported in 79 patients in the literature to date. The majority of these patients were affected by sarcoidosis and developed non-Hodgkin lymphoma or acute leukemia after 1-2 years; however, in <20 cases the malignancy developed first. This report presents the case of an 83-year-old male with a clinical history of Helicobacter pylori-positive gastric mucosa-associated lymphoid tissue lymphoma. The patient developed sarcoidosis 10 years after the first diagnosis, which caused the diagnostic work-up and differential diagnosis between a lymphoma relapse and de novo sarcoidosis to be challenging
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