Fig. 1
Adapted from the HLH Center of Excellence at Cincinnati Children’s Hospital Medical Center (original algorithm can be downloaded here https://www.cincinnatichildrens.org/service/h/hlh/clinical/test)
A proposed algorithm for evaluation and management of suspected HLH. It must be noted that there remains insufficient data to make definitive recommendations regarding diagnosis and management of HLH and the above algorithm is based on author consensus. Above all it must be emphasized that patients with hyper-inflammatory phenotypes of unclear etiology should undergo exhaustive workup for malignancy, infection, and rheumatologic disease, before etoposide-based therapy is considered. *Anti-inflammatory therapies may include agents such as steroids and/or cytokine-directed agents (such as anakinra, tocilizumab, emapalumab, or ruxolitinib).