GÉTICA 2021

32 VII FORO DE I nmunología Traslacional e INMUNOTERAPIA DEL CÁNCER the patient was performed confirming the syn- drome with hemophagocytic lymphohistiocy- tosis affecting the bone marrow, lymph nodes, liver and spleen (Fig. 2) and also the central ner- vous system. from authorised investigations. Due to the death of our patient, informed consent was obtained from his next of kin, his daughter. All previously published reports were accessed from indexed medical journals, and safety signals gathered from public access pharmacovigilance databases. As far as we know, there are no case reports about sHLH caused by atezolizumab, and only twelve individu- al safety reports registered in the WHO Programme for International Drug Monitoring database (4), all from 2018 to this moment, together with fifteen (2 in 2019, 10 in 2020, 3 in 2021 until now) in the EUdra database, which includes our report. Results and conclusions: With the increasing use of novel agents such as checkpoint inhibitors, the toxicity profile of our drugs has been changed and uncommon syndromes are more frequent nowadays. Besides there are more common in patients with comorbidity or with drug combi- nations. Recently sHLH has been described in patients receiving ICI therapy more frequently (Table I). Early intervention is critical to stop pro- gression and to improve the patient’s condition, so premature initiation of steroid generally carries a favorable prognosis. Most patients will respond during the first 48-72 hours. Still, early diagnosis may be difficult to make with 100 % certainty (5). Lastly in cases where there is no improvement with steroids, aggressive supportive management is necessary with intensification of therapy, fol- lowing multi-immune suppressive drug protocols and adding interleukins targeted therapies (6,7), with previous poor results reported (Table I), like in our case. While the potential impact of such procedures is recognized, an optimal regimen se- quence has still to be found and a deep study of the causes together with the development of spe- cific protocols for these patients is necessary. Figure 2. Biomarkers and key features are needed to better identify the patients at risk of developing severe immune-related adverse events. Also, a higher de- gree of suspicion and early intervention is need- ed to improve outcomes and survival in acquired HLH, especially with the increasingly frequent use of immune checkpoint inhibitors and the devel- opment of new immune agents (3). The contin- uous characterization of this new adverse event and its care would also redefine their safety pro- file, having implications for the future update of guidelines for the management of immunothera- py-related toxicities. Patients and method: Our case is the first de- scription of secondary hemophagocytic lympho- histiocytosis, with the use of atezolizumab. We provide a clinical and literature review of this rare toxicity with the use of immune checkpoint inhib- itors. The case information was obtained from the patient´s medical history records, images were