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.