Salvage therapy for refractory hemophagocytic lymphohistiocytosis: A review of the published experience. Pediatr Blood Cancer 2017 Apr;64(4)
Date
10/28/2016Pubmed ID
27786410DOI
10.1002/pbc.26308Scopus ID
2-s2.0-84999791337 (requires institutional sign-in at Scopus site) 40 CitationsAbstract
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.
Author List
Marsh RA, Jordan MB, Talano JA, Nichols KE, Kumar A, Naqvi A, Vaiselbuh SR, Histiocyte Society Salvage Therapy Working GroupAuthor
Julie-An M. Talano MD Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Drug Resistance, NeoplasmDrug Therapy, Combination
Humans
Lymphohistiocytosis, Hemophagocytic
Prognosis
Salvage Therapy