Fatal Hemophagocytic Lymphohistiocytosis Resulting in the Death of a Previously Healthy 49-Year-Old Man in Ten Days.

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive, life-threatening condition with sudden onset of a systemic inflammatory response syndrome. The triggers can be apparently non-specific, and the clinical presentations can be very deceptive during a rapidly deteriorating clinical course. Herein, we report a case of a 49-year-old White/Caucasian male with no known past medical history who presented with multi-organ failure, including liver, kidney, and bone marrow, along with disseminated intravascular coagulation. He had a high probability of HLH. Unfortunately, he died ten days after the initial presentation. At autopsy, the liver was necrotic and immunostains revealed diffuse positivity for HSV-1 & 2. The bone marrow was markedly hypocellular with phagocytes containing intact and fragmented red blood cells. There was also disseminated fungal infection involving almost all tissues. PCR, done on frozen tissue samples, revealed Aspergillus fumigatus. The rapid and fatal course of this patient illustrates the sometimes-aggressive course of HLH and the importance of autopsy examination in revealing the underlying etiology for this patient's death.