Surviving Grade 4 ICANS: A Case Report and Discussion of Emerging Management Strategies

Chimeric antigen receptor (CAR) T-cell therapy is increasingly used in both oncologic and nononcologic conditions. Although low-grade neurotoxicity may be easily treated, severe neurotoxicity remains clinically challenging and many times fatal. We present the case of a young adult with relapsed acute lymphoblastic leukemia who developed grade 4 immune effector cell–associated neurotoxicity syndrome (ICANS), manifesting as severe cerebral edema refractory to first-line therapies. We share our treatment strategy consisting of multiple lines of targeted immunotherapy and proactive intracranial pressure monitoring. The patient survived and continues to remain in remission with meaningful neurologic recovery. Because of the growing use of chimeric antigen receptor T-cell therapies, neurologists need to be aware and ready to treat these potentially fatal cases.