IMPORTANCE OF HYPERBILURUBINEMIA IN DIFFERENTIATION OF PRIMARY AND SECONDARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN PEDIATRIC CASES

seval ozen, alper Dai, enes coskun, serdar oztuzcu, sercan ergun, elif aktekin, sibel yavuz, Ali Bay
  • seval ozen
    Gaziantep Universitesi Tıp Fakultesi Cocuk Hastalıkları Klinigi, Gaziantep, Turkey, Turkey
  • alper Dai
    Affiliation not present
  • enes coskun
    Affiliation not present
  • serdar oztuzcu
    Affiliation not present
  • sercan ergun
    Affiliation not present
  • elif aktekin
    Affiliation not present
  • Ali Bay
    Gaziantep Universitesi Tıp Fakultesi Cocuk Hastalıkları Klinigi, Gaziantep, Turkey, Turkey

Abstract

Background and 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.

Patient and Method: 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).

Conclusion: We showed that cholestasis and hyperbilurubinemia findings of HLH patients at the initial diagnosis should be considered in favor of primary HLH and increased level of CRP should be considered in favor of secondary HLH.

Keywords

Hemophagocytic lymphohistiocytosis; cholestasis; child, hyperbilurubinemia.

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Submitted: 2014-08-29 14:22:41
Published: 2014-10-28 00:00:00
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