Hemophagocytic lymphohistiocytosis: new grading classification and targeted therapies. (PubMed, Clin Adv Hematol Oncol)
Although etoposide with dexamethasone remains the most frequently used first-line regimen, various new therapies are now being employed in the management of HLH. The interferon gamma inhibitor emapalumab, the Janus kinase signal transducer and activator of transcription pathway inhibitor ruxolitinib, and the interleukin 6 (IL-6) inhibitor tocilizumab have been trialed in HLH management, with additional treatment options being inhibition of IL-18 and tumor necrosis factor alpha. Here, we summarize current management options for HLH; we also propose a new grading system for HLH based on Common Terminology Criteria for Adverse Events version 5.0 as well as on known prognostic factors (eg, abnormal bilirubin and transaminase levels, elevated creatinine level, respiratory failure, neutropenia, hypertriglyceridemia, hypoalbuminemia, and coagulopathy), which could standardize the diagnosis and guide prompt and appropriate management.