Splenic Nodular Sclerosis-Variant Hodgkin Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: A Diagnostic Challenge in an Adult Patient.

Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome, is a life-threatening hyperinflammatory syndrome. Primary HLH is more common in children, while secondary HLH, triggered by malignancy, infection, or autoimmunity, predominates in adults. We report the case of a 56-year-old man referred to our institution with a 10-month history of fever (>38.3°C) meeting diagnostic criteria for fever of unknown origin (FUO). Clinical evaluation revealed splenomegaly and pancytopenia. Given the high clinical suspicion of HLH, an H-score of 219 points was calculated, and management was initiated. As part of the diagnostic workup, Ga-67 citrate scintigraphy and multimodal imaging were performed, revealing intense splenic uptake and structural abnormalities. These findings guided a targeted splenectomy, which confirmed classic splenic Hodgkin lymphoma (HL) (nodular sclerosis subtype). This case illustrates a systematic clinical approach to HLH, enabling the identification of the underlying etiology and facilitating timely intervention. The association between HLH and HL is rare and carries a high mortality rate; however, prompt etiological treatment significantly improves survival. We present, to the best of our knowledge, the first-ever reported case of HLH associated with splenic HL.