The novel use of emapalumab and ruxolitinib in acquired
malignancy-associated hemophagocytic lymphohistiocytosis in pediatric
patients
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal syndrome
of immune dysregulation that is classified into primary and secondary
forms. The standard of care is established with dexamethasone and
etoposide, but there are currently no guidelines for refractory HLH or
cases triggered by infection or malignancy. [1] We describe here a
series of pediatric patients with malignancy-associated HLH (m-HLH) to
discuss the complexities in the initial diagnostic considerations, the
balance of therapeutic regimens and their toxicities, and the novel use
of emapalumab and ruxolitinib in patients with refractory disease.