title: Secondary Hemophagocytic Lymphohistiocytosis: Do We Really Need Chemotherapeutics for All Patients?
abstract: Because of the acute and life-threatening course of the hemophagocytic lymphohistiocytosis (HLH) syndrome, International Histiocyte Society guidelines recommend chemoimmune therapy for the treatment of both primary and secondary HLH (sHLH). To manage children with sHLH, instead of HLH-2004 protocol we considered less immunosuppressive/cytotoxic approach. We assessed 12 children who fulfilled the diagnostic criteria for sHLH between January 2009 and March 2015. Multivariate Cox regression analysis showed that ferritin levels (hazard ratio=1.02, P=0.006), pediatric logistic organ dysfunction scores (hazard ratio=1.01, P=0.001) were the predictors of the survival. The hospital survival was 83% for patients with sHLH who were treated with less immunosuppressive therapy. In conclusion initiation of HLH-specific therapy for the patients with hyperferritinemia-associated sHLH should be delayed while awaiting resolution of systemic inflammation with less immunosuppressive therapy.
authors.affiliation: *Department of Pediatrics, Cukurova University Faculty of Medicine †Department of Pediatrics, Division of Pediatric Oncology, Adana Teaching and Medical Research Center, Baskent University Faculty of Medicine, Adana, Turkey.
authors.forename: Zeliha
authors.initials: Z
authors.lastname: Haytoglu
authors.forename: Nalan
authors.initials: N
authors.lastname: Yazici
authors.forename: Ayse
authors.initials: A
authors.lastname: Erbay
mesh_terms: D015415:Biomarkers; D002648:Child; D002675:Child, Preschool; D003603:Cytotoxins / Q000627:therapeutic use*; D005260:Female; D005293:Ferritins / Q000097:blood; D006760:Hospitalization; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000188:drug therapy* / Q000401:mortality; D008297:Male; D000069342:Medical Overuse / Q000517:prevention & control*; D063305:Organ Dysfunction Scores; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D003603:Cytotoxins; D007166:Immunosuppressive Agents; D005293:Ferritins