Salvage therapy for refractory hemophagocytic lymphohistiocytosis: A review of the published experience

Pediatr Blood Cancer. 2017 Apr;64(4). doi: 10.1002/pbc.26308. Epub 2016 Oct 27.


Hemophagocytic lymphohistioytosis (HLH) is a severe, life-threatening hyperinflammatory disorder that requires prompt diagnosis and treatment. Approximately, 25-50% of patients with HLH fail to achieve remission with established regimens that include dexamethasone and etoposide, or methylprednisolone and antithymocyte globulin (ATG). Some of these patients may require salvage or alternative therapeutic approaches. There is a paucity of literature regarding effective salvage therapies for patients with refractory HLH. In this review, we summarize the published experience of four therapeutics reported for using at least two patients with HLH refractory to dexamethasone and etoposide or methylprednisolone and ATG.

Keywords: HLH; hemophagocytic lymphohistiocytosis; refractory HLH; salvage therapy.

Publication types

  • Review

MeSH terms

  • Drug Resistance, Neoplasm / drug effects*
  • Drug Therapy, Combination
  • Humans
  • Lymphohistiocytosis, Hemophagocytic / drug therapy*
  • Prognosis
  • Salvage Therapy*