Treatment

How We Treat Hemophagocytic Lymphohistiocytosis

Treatment should be started immediately once a diagnosis of HLH is established. Treatment includes dexamethasone and etoposide. Alternative “salvage” therapies that target T cells include anti-thymocyte globulin and alemtuzumab.

While patients are undergoing treatment, efforts should be made to determine if there is a genetic defect present. Screening tests can provide early data that helps guide therapy while confirmative genetic testing is still pending. If a genetic defect is observed, curative treatment with allogeneic hematopoietic cell transplantation should be considered. In the absence of a genetic diagnosis, transplant should also be considered for young patients, those with a family history of bad inflammation / death, recurrent clinical phenotype, or functional laboratory data suggesting an inherited defect in lymphocyte cytotoxicity.


Gene mutation