title: Importance of hyperbilirubinemia in differentiation of primary and secondary hemophagocytic lymphohistiocytosis in pediatric cases.
abstract: OBJECTIVE
  Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyper-inflammatory disease. It is difficult to differentiate between primary and secondary HLH based on clinical findings at the onset of disease. We aimed to find parameters that can help to differentiate primary and secondary HLH at initial diagnosis especially for physicians working in developing countries.
  
  
  METHODS
  We retrospectively analyzed data of 38 HLH patients who were admitted to the Pediatric Hematology Department of Gaziantep University between January 2009 and December 2013.
  
  
  RESULTS
  Of 38 patients, 20 were defined as primary, and 18 were secondary HLH. The average age of primary and secondary HLH patients was 31±9 and 81±14 months, respectively (p=0.03). We found consanguinity rates significantly higher in primary HLH patients compared to secondary HLH patients (p=0.03). We found that total and direct bilirubin levels significantly increased in primary HLH patients compared to secondary HLH patients (p=0.006, p=0.044). Also, CRP levels were found markedly increased in secondary HLH patients compared to primary ones (p=0.017).
  
  
  CONCLUSIONS
  We showed that cholestasis and hyperbilirubinemia findings of HLH patients at the initial diagnosis should be considered in favor of primary HLH, and an increased level of CRP should be considered in favor of secondary HLH.
authors.affiliation: Gaziantep University Department of Pediatrics, Gaziantep, Turkey.
authors.forename: Seval
authors.initials: S
authors.lastname: Ozen
authors.affiliation: Gaziantep University Department of Pediatrics, Gaziantep, Turkey.
authors.forename: Alper
authors.initials: A
authors.lastname: Dai
authors.affiliation: Gaziantep University Department of Pediatrics, Gaziantep, Turkey.
authors.forename: Enes
authors.initials: E
authors.lastname: Coskun
authors.affiliation: Gaziantep University Department of Medical Biology, Gaziantep, Turkey.
authors.forename: Serdar
authors.initials: S
authors.lastname: Oztuzcu
authors.affiliation: Gaziantep University Department of Medical Biology, Gaziantep, Turkey.
authors.forename: Sercan
authors.initials: S
authors.lastname: Ergun
authors.affiliation: Gaziantep University Department of Pediatrics, Gaziantep, Turkey.
authors.forename: Elif
authors.initials: E
authors.lastname: Aktekin
authors.affiliation: Gaziantep University Department of Pediatrics, Gaziantep, Turkey.
authors.forename: Sibel
authors.initials: S
authors.lastname: Yavuz
authors.affiliation: Gaziantep University Department of Pediatrics, Gaziantep, Turkey ; Gaziantep University Division of Pediatric Hematology Gaziantep, Turkey.
authors.forename: Ali
authors.initials: A
authors.lastname: Bay