Here we review the pathophysiology and management of cytokine release
syndrome (CRS) secondary to immunotherapy, and potential options for CRS
refractory to IL6 inhibition and glucocorticoids, for which there are no
proven treatments. To illustrate, we describe a patient with B-cell acute
lymphoblastic leukemia who developed refractory grade 4 CRS following
CD19-directed chimeric antigen receptor T-cell therapy, treated with
tocilizumab, methylprednisolone, siltuximab, and the IFNγ inhibitor
emapalumab, with complete remission from leukemia for 12 months. See
related article by Bailey et al., p. 136 (15).