title: Salvage therapy for refractory hemophagocytic lymphohistiocytosis: A review of the published experience.
abstract: 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.
authors: Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital, Cincinnati, Ohio.
authors: Rebecca A
authors: RA
authors: Marsh
authors: Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital, Cincinnati, Ohio.|Division of Immunobiology, Cincinnati Children's Hospital, Cincinnati, Ohio.
authors: Michael B
authors: MB
authors: Jordan
authors: Division of Pediatric Hematology and Oncology Medical College of Wisconsin, Milwaukee, Wisconsin.
authors: Julie-An
authors: JA
authors: Talano
authors: Division of Cancer Predisposition, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors: Kim E
authors: KE
authors: Nichols
authors: Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital, Cincinnati, Ohio.
authors: Ashish
authors: A
authors: Kumar
authors: Division of Haematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario.
authors: Ahmed
authors: A
authors: Naqvi
authors: Children's Cancer Center, Staten Island University Hospital at Northwell Health, Staten Island, New York.
authors: Sarah R
authors: SR
authors: Vaiselbuh
mesh_terms: D019008:Drug Resistance, Neoplasm / Q000187:drug effects*; D004359:Drug Therapy, Combination; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy*; D011379:Prognosis; D016879:Salvage Therapy
keywords: HLH; hemophagocytic lymphohistiocytosis; refractory HLH; salvage therapy