Hepatosplenic T-cell lymphoma with hemophagocytic lymphohistiocytosis, revealed by fever of unknown origin

The unusual peripheral lymphoma known as hepatosplenic T‑cell lymphoma (HSTCL) is a rare hematologic cancer. In this report, we discuss a case of HSTCL with hemophagocytic lymphohistiocytosis that was discovered due to a prolonged fever in a 27-year-old woman with no prior medical history. Infectious and autoimmune diseases were ruled out during the etiological review. Histopathological analysis of the bone marrow revealed hemophagocytosis. A diagnostic splenectomy was performed after the patient’s clinical condition worsened, showing no response to corticosteroids, and notable splenomegaly and hepatomegaly were observed on the positron emission tomography (PET) scan. The diagnosis of HSTCL was confirmed through pathological examination of the surgical specimens. In addition, a complementary genetic analysis revealed a gamma-delta T‑cell lymphoma. Hemophagocytic lymphohistiocytosis remains a diagnostic and therapeutic challenge, and physicians should always consider the possibility of an underlying hematologic disorder. Our case highlights the difficulties in diagnosing HSTCL due to the various possible causes of prolonged fever of unknown origin, as well as the nonspecific clinical presentation and the absence of peripheral lymphadenopathy. Our report discusses a new case of HSTCL associated with hemophagocytic lymphohistiocytosis to raise awareness among physicians about this rare entity. This association can be confusing and may lead to delays in diagnosis and thus worsening prognosis.