Hemophagocytic Lymphohistiocytosis Masquerading as Acute Liver Failure : A Single Center Experience
BACKGROUND/AIM: Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening disorder of extreme inflammation and unregulated immune response which require prompt recognition and early introduction of definitive therapy. HLH can present with wide range of hepatic dysfunction ranging from mild elevation of transaminases to liver failure. This study is carried out to describe the clinical and laboratory presentation of HLH.
METHODS: Patients who were diagnosed with HLH between January 2013 and December 2015 were retrospectively included in this study.
RESULTS: Six patients were diagnosed as secondary HLH with median age of 28.5 years at diagnosis. All patients were presented with history of deep jaundice and high grade fever with pancytopenia and splenomegaly. Underlying diagnosis was viral infections in 4 and probable viral infection in remaining two. Bone marrow hemophagocytosis was present in 3 cases. Three patients were treated with corticosteroids only and one each with corticosteroids with cyclosporine or intravenous immunoglobulin (IVIG) and HLH treatment protocol. One patient died due to acute respiratory distress syndrome (ARDS); another patient died in follow-up due to respiratory failure due to pneumonia.
CONCLUSIONS: HLH is rare and potentially life-threatening cause of prolonged fever, jaundice and pancytopenia. Early diagnosis and initiation of specific therapy can improve clinical outcome.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2017 |
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| Erschienen: |
2017 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:7 |
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| Enthalten in: |
Journal of clinical and experimental hepatology - 7(2017), 3 vom: 15. Sept., Seite 184-189 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Jagtap, Nitin [VerfasserIn] |
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| Links: |
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| Anmerkungen: |
Date Revised 30.09.2020 published: Print-Electronic Citation Status PubMed-not-MEDLINE |
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| doi: |
10.1016/j.jceh.2017.01.119 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM276404122 |
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| 245 | 1 | 0 | |a Hemophagocytic Lymphohistiocytosis Masquerading as Acute Liver Failure |b A Single Center Experience |
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| 500 | |a Date Revised 30.09.2020 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status PubMed-not-MEDLINE | ||
| 520 | |a BACKGROUND/AIM: Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening disorder of extreme inflammation and unregulated immune response which require prompt recognition and early introduction of definitive therapy. HLH can present with wide range of hepatic dysfunction ranging from mild elevation of transaminases to liver failure. This study is carried out to describe the clinical and laboratory presentation of HLH | ||
| 520 | |a METHODS: Patients who were diagnosed with HLH between January 2013 and December 2015 were retrospectively included in this study | ||
| 520 | |a RESULTS: Six patients were diagnosed as secondary HLH with median age of 28.5 years at diagnosis. All patients were presented with history of deep jaundice and high grade fever with pancytopenia and splenomegaly. Underlying diagnosis was viral infections in 4 and probable viral infection in remaining two. Bone marrow hemophagocytosis was present in 3 cases. Three patients were treated with corticosteroids only and one each with corticosteroids with cyclosporine or intravenous immunoglobulin (IVIG) and HLH treatment protocol. One patient died due to acute respiratory distress syndrome (ARDS); another patient died in follow-up due to respiratory failure due to pneumonia | ||
| 520 | |a CONCLUSIONS: HLH is rare and potentially life-threatening cause of prolonged fever, jaundice and pancytopenia. Early diagnosis and initiation of specific therapy can improve clinical outcome | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a ARDS, acute respiratory distress syndrome | |
| 650 | 4 | |a CMV, cytomegalovirus | |
| 650 | 4 | |a CSA, cyclosporine | |
| 650 | 4 | |a EBV, Epstein Barr virus | |
| 650 | 4 | |a HLH, hemophagocytic lymphohistiocytosis | |
| 650 | 4 | |a IVIG, intravenous immunoglobulin | |
| 650 | 4 | |a MAS, macrophage activation syndrome | |
| 650 | 4 | |a acute liver failure | |
| 650 | 4 | |a hepatotoxicity | |
| 650 | 4 | |a viral hepatitis | |
| 700 | 1 | |a Sharma, Mithun |e verfasserin |4 aut | |
| 700 | 1 | |a Rajesh, Gupta |e verfasserin |4 aut | |
| 700 | 1 | |a Rao, Padaki Nagaraja |e verfasserin |4 aut | |
| 700 | 1 | |a Anuradha, Sekaran |e verfasserin |4 aut | |
| 700 | 1 | |a Tandan, Manu |e verfasserin |4 aut | |
| 700 | 1 | |a Ramchandani, Mohan |e verfasserin |4 aut | |
| 700 | 1 | |a Reddy, Duvvuru Nageshwar |e verfasserin |4 aut | |
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