Nasopharyngeal Carcinoma Associated with Hemophagocytic Lymphohistiocytosis

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare disease with high fatality. It can be genetic, or it can be associated with infection, malignancy or autoimmune disorders. Cases of nasopharyngeal carcinoma (NPC) associated with HLH are rare. Case Report: A 56 year-old man with recurrent NPC with metastases had concurrent HLH. The patient presented with fever, splenomegaly, cytopenia in three lineages, elevated ferritin, elevated liver transaminases, hyperbilirubinemia, and hemophagocytosis in bone marrow. He also had a very high serum Epstein-Barr (EB) viral load. We diagnosed HLH in this patient, believing it to be associated with either EB virus or NPC. Following treatment with etoposide, cisplatin and intravenous immunoglobulin, the levels of liver transaminases and bilirubin decreased, but the patient's pancytopenia did not improve before he died of aspiration pneumonia 6 months after the diagnosis of NPC. Conclusions: HLH is a disease with high fatality. Whenever there is a degree of clinical suspicion for HLH, etoposide-based chemotherapy should be started as soon as possible, and the underlying causes of the disease should be treated. We report this patient with NPC and HLH, which can be associated with either EBV or NPC. We treated both etiologies, and the patient's symptoms and liver function improved. The interactions between EBV, NPC, and HLH are interesting and merit further investigation.