Treatment of refractory hemophagocytic lymphohistiocytosis with emapalumab despite severe concurrent infections

Dana T. Lounder, Qiong Bin, Cristina de Min and Michael B. Jordan

Article Figures & Data


  • Figure 1.

    HLH disease features and viremias after treatment with emapalumab. (A-B) Absolute neutrophil and platelet counts, both heavily supported by transfusion until about day 21. (C-D) Fibrinogen (supported by fresh-frozen plasma transfusion until day 21) and D-dimer levels. (E-F) Alanine aminotransferase (ALT) and direct bilirubin levels. (G-H) The inflammatory markers ferritin and soluble interleukin-2 receptor (sIL2r [sCD25]). Reported ferritin values are limited to 40 000 μg/L by the clinical laboratory. Ferritin values decreased to <500 μg/L by day 180. (I-J) Blood levels of EBV and CMV. Arrows on the x-axis of each graph indicate the start of emapalumab treatment, and initial and final values for each marker are shown.

  • Figure 2.

    IFN-γ–associated chemokines after treatment with emapalumab. The levels of CXCL9 (A) and CXCL10 (B), 2 chemokines induced by IFN-γ, after initiation of therapy with emapalumab. Baseline and final levels for each biomarker are indicated.