Treatment-related hemophagocytic lymphohistiocytosis due to atezolizumab: a case report and review of the literature

Abstract

Background Immune checkpoint inhibitors avoid inhibition of T-cell responses, upregulating antitumor immune response. Moreover, a dysregulation with hyperactive immune response can be caused, some of them underdiagnosed. Hemophagocytic lymphohistiocytosis is a rare and often fatal syndrome of uncontrolled and ineffective hyperinflammatory response that triggers an inflammatory cascade that can lead in many cases to death. Case presentation We report the case of a 67-year-old Caucasian man with stage IV lung adenocarcinoma who developed hemophagocytic lymphohistiocytosis after initiation of atezolizumab, an antagonist of programmed death-ligand 1. Even with early diagnosis and proper treatment, death occurs in approximately half of all cases reported. Conclusion Key markers are needed to better identify patients at risk of developing severe immune-related adverse events. In addition to key markers, a higher degree of suspicion and early intervention are needed to improve outcomes in acquired hemophagocytic lymphohistiocytosis, especially with the increasingly and expanding use of immune activation.

A Case Report of Hemophagocytic Lymphohistiocytosis and Meningitis Due to Atezolizumab Treatment for Lung Adenocarcinoma

“…Therefore, the combination of HLH and meningitis due to atezolizumab is very rare, making this case very valuable. To the best of our knowledge, there have been two previous case reports of HLH due to atezolizumab, with two cases reported [ 12 , 13 ]. Due to the small number of reports of atezolizumab-induced HLH, the predisposing factors for this disease have not yet been clearly identified.…”

“…All patients were treated with steroids, including six with methylprednisolone (mPSL) pulse, four with etoposide, and one with tocilizumab. One patient died; others were discharged with mild symptoms (Table 2 ) [ 12 , 13 , 15 - 21 ].…”

Low interleukin-10 level indicates a good prognosis in Salmonella enterica serovar typhimurium-induced pediatric hemophagocytic lymphohistiocytosis: A case report

“…There is no consensus on how to treat S. typhimurium -triggered sHLH, and early intervention is needed to improve outcomes in patients with HLH[ 18 ]. Most of the current research is empirical, and the decision-making process is relevant to the time point at which positive culture results are obtained and based on the clinician's experience.…”

Hemophagocytic lymphohistiocytosis/cytokine release syndrome secondary to neoadjuvant pembrolizumab for triple-negative breast cancer: a case study

“…Some patients were treated with the HLH-2004 protocol ( 22 , 27 , 31 , 43 , 44 ), while others were treated with corticosteroids alone ( 23 , 24 , 26 , 28 , 32 , 35 , 45 – 47 ). Some centers utilized combinations of corticosteroids and other agents such as intravenous immunoglobulin (IVIG) ( 29 , 48 ), mycophenolate mofetil (MMF) and cyclosporin ( 30 ), anakinra ( 29 , 49 ), tocilizumab ( 31 , 50 ), and infliximab ( 25 ). The management of CRS from immune checkpoint inhibition also varied among published case studies, from high-dose steroids alone ( 16 , 17 , 20 , 21 ) to combination therapy with steroids, MMF, plasma exchange, and IVIG ( 18 ).…”