Patient 2: A.M.
A 16-year-old female with persistent EBV-driven NK/T-cell lymphoma of the nasopharynx presented in septic shock with pancytopenia and disseminated intravascular coagulation. Initial diagnostic considerations included progressive lymphoma, bacteremia, and EBV viremia. HLH was a consideration,
but she did not meet criteria until hospital day 7. Treatment with dexamethasone, etoposide, and rituximab with her persistent EBV infection showed minimal effect. On day 17, a bone marrow biopsy showed evidence of NK/T-cell lymphoma and gemcitabine and oxaliplatin were started, but she developed acute decompensation with hemodynamic instability and multi-organ system failure likely due to fulminant EBV viremia. Her systemic inflammation worsened as evidenced by a rise in her ferritin, sIL-2 receptor, and CXCL9, a biomarker of IFNγ. [4] We held treatment for her lymphoma to reduce toxicities and better manage her HLH. With continued clinical deterioration, compassionate use of emapalumab, an IFNγ inhibitor indicated in primary HLH, led to dampening of her systemic inflammatory processes with resolution of fevers, decreased inflammatory markers, and signs of recovery of her hepatobiliary, renal, and gastrointestinal systems. [5] Unfortunately, despite demonstrating response to emapalumab, our patient succumbed to an intraparenchymal hemorrhage after developing fulminant candidiasis while on treatment dosing of micafungin.