A fatal case of hemophagocytic lymphohistiocytosis in an elderly male

Abstract

We present a case of an 87-year-old male with hemophagocytic lymphohistiocytosis (HLH). Hemophagocytic lymphohistiocytosis is a life-threatening disorder characterized by dysregulated immune activity leading to end-organ damage. Hematologic malignancies appear to be the main cause of secondary HLH. Diagnostic criteria include fever, cytopenia, splenomegaly, hypertriglyceridemia, hypofibrinogenemia, a ferritin level greater than 500 ng/mL, low NK-cell activity, and elevated sIL2Ra levels greater than or equal to 2400 U/mL. Five out of eight of the criteria are necessary to establish a diagnosis of HLH. Our patient met five out of eight diagnostic criteria for HLH (fever, cytopenia, splenomegaly, hypertriglyceridemia, and ferritin level greater than 500 ng/mL). Bone marrow aspirate revealed hemophagocytosis. Bone marrow biopsy revealed marginal zone B cell lymphoma, which is presumed to be the underlying cause of his condition. Due to poor functional status, the patient was a poor candidate for curative treatment, and the family chose not to pursue this route. The patient was treated with Decadron 20 mg intravenous (IV) push daily until he ultimately died, likely due to respiratory failure due to heart failure (HF).