Importance of hyperbilirubinemia in differentiation of primary and secondary hemophagocytic lymphohistiocytosis in pediatric cases
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 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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2014 |
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| Erschienen: |
2014 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:6 |
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| Enthalten in: |
Mediterranean journal of hematology and infectious diseases - 6(2014), 1 vom: 13., Seite e2014067 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Ozen, Seval [VerfasserIn] |
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| Anmerkungen: |
Date Completed 21.11.2014 Date Revised 10.11.2023 published: Electronic-eCollection Citation Status PubMed-not-MEDLINE |
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| doi: |
10.4084/MJHID.2014.067 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM243650892 |
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| 245 | 1 | 0 | |a Importance of hyperbilirubinemia in differentiation of primary and secondary hemophagocytic lymphohistiocytosis in pediatric cases |
| 264 | 1 | |c 2014 | |
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| 500 | |a Date Completed 21.11.2014 | ||
| 500 | |a Date Revised 10.11.2023 | ||
| 500 | |a published: Electronic-eCollection | ||
| 500 | |a Citation Status PubMed-not-MEDLINE | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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) | ||
| 520 | |a CONCLUSION: 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 | ||
| 650 | 4 | |a Journal Article | |
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| 700 | 1 | |a Coskun, Enes |e verfasserin |4 aut | |
| 700 | 1 | |a Oztuzcu, Serdar |e verfasserin |4 aut | |
| 700 | 1 | |a Ergun, Sercan |e verfasserin |4 aut | |
| 700 | 1 | |a Aktekin, Elif |e verfasserin |4 aut | |
| 700 | 1 | |a Yavuz, Sibel |e verfasserin |4 aut | |
| 700 | 1 | |a Bay, Ali |e verfasserin |4 aut | |
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