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| 1 | Acute liver failure secondary to severe systemic disease from fatal hemophagocytic lymphohistiocytosis:Case report and systematic literature review显示文摘AIM To systematically review liver disease associated with hemophagocytic lymphohistiocytosis(HLH),propose reasonable contraindications for liver transplantation for liver failure in HLH,and report an illustrative case.METHODS Systematic review according to PRISMA guidelines of hepatic manifestations of HLH using computerizedliterature search via PubMed of articles published since 1980 with keywords('hemophagocytic lymphohistiocytosis' or 'HLH') AND('liver' or 'hepatic'). Two authors independently performed literature search and incorporated articles into this review by consensus. Illustrative case report presented based on review of medical chart,and expert re-review of endoscopic photographs,radiologic images,and pathologic slides. RESULTS A 47-year-old Caucasian male,was hospitalized with high-grade pyrexia,rash,total bilirubin = 45 g/dL,moderately elevated hepatic transaminases,ferritin of 3300 ng/dL,leukopenia,and profound neutropenia(absolute neutrophil count < 100 cells/mm3). Viral serologies for hepatitis A,B,and C were negative. Abdominal computed tomography scan and magnetic resonance imaging revealed no hepatic or biliary abnormalities. Pathologic analysis of liver biopsy revealed relatively well-preserved hepatic parenchyma without lymphocytic infiltrates or macrophage invasion,except for sparse,focal hepatocyte necrosis. Bone marrow biopsy and aspirate revealed foamy macrophages engulfing mature and precursor erythrocytes,consistent with HLH. Interleukin-2 receptor(CD25) was highly elevated,confirming diagnosis of HLH according to Histiocytic Society criteria. Patient initially improved after high-dose prednisone therapy. Patient was judged not to be a liver transplant candidate despite model for end stage liver disease(MELD) score = 33 because liver failure was secondary to severe systemic disease from HLH,including septic shock,focal centrilobular hepatocyte necrosis from hypotension,bone marrow failure,and explosive immune activation from HLH. The patient eventually succumbed to overwhelming sepsis,progressive liver failure,and disseminated intravascular coagulopathy. Systematic review reveals liver injury is very common in HLH,and liver failure can sometimes occur. Data on liver transplantation for patients with HLH are very limited,and so far the results have shown a generally much worse prognosis than for other liver transplant indications. Liver transplantation should not be guided solely by MELD score,but should include liver biopsy results and determination whether liver failure is from intrinsic liver injury vs multisystem(extrahepatic) organ failure from HLH.CONCLUSION This case report illustrates that liver transplantation may not be warranted when liver failure associated with HLH is primarily from multisystem failure from HLH. Liver biopsy may be very helpful in determining the severity and pathophysiology of the liver disease. | Mitchell S Cappell Ismail Hader Mitual Amin | 2018 | World Journal of Hepatology2018，10，9： | 5 |
| 2 | ELDONET- European Light Dosimeter Network hardware and software 显示文摘 | Hader D P Lebert M Marangoni R | 1999 | J Photochem Photobiol B: Biol1999，52，1： | 1 |
| 3 | Primitive neuroectodermal tumors in the central nervous system following cranial i rradiation: a report of four cases 显示文摘 | Hader W J Drovini-Zis K Maguire JA | 2003 | Can- cer2003，97，4： | 1 |
| 4 | Your hand in pain management 显示文摘 | Hader CF Guy J | 2004 | Nurs Man- age2004，35，11： | 1 |
| 5 | Mesenchymal-epithelial transition in epithelial response to injury: The role of Foxc2显示文摘 | Hader C Marlier A Cantley L | 2010 | Oncogene2010，29，7： | 1 |
| 6 | UV - B - induced formation of re-active oxygen species and oxidative damage of the cyanobacteriun Anabaena sp : protective effects of ascorbic acid and N - acetyl - L - cysteine 显示文摘 | He YY Hader DP | 2002 | J Photochem Photobiol B : Biology2002，66，： | 1 |
| 7 | Effects of increased solar ultraviolet radiation on aquatic ecosystems显示文摘 | HADER D P WORREST R C KUMAR H D | 1995 | Ambio1995，24，： | 1 |
| 8 | Surgical management of hypothalamic hamartomas in patients with gelasticepilepsy 显示文摘 | Addas B Sherman EM Hader WJ | 2008 | N eurosurg Focus2008，25，3： | 1 |
| 9 | Effects of solar UV-B radiation on aquatic ecosystems显示文摘 | HADER D P | 2000 | Advance in Space Research2000，26，12： | 1 |
| 10 | Risk of enhanced solar ultraciolet radiation for aquatic ecosystems显示文摘 | Hader D P | 1993 | Prog Phyeol Res1993，9，： | 1 |
| 11 | Methylotrophic yeasts as factories for the production of foreign proteins 显示文摘 | Faber K N Hader W Ab G | 1995 | Yeast1995，11，14： | 1 |
| 12 | Workplace violence survey 2008 unsettling findings: Employees safety isn't the norm in our healthcare settings显示文摘 | Hader R | 2008 | Nurs Manage2008，39，7： | 1 |
| 13 | Complications of epilepsy surgery—A systematic review of focal surgical resections and invasive EEG monitoring显示文摘 | Walter J. Hader Jose Tellez‐Zenteno Amy Metcalfe Lisbeth Hernandez‐Ronquillo Samuel Wiebe Churl‐Su Kwon Nathalie Jette | 2013 | Epilepsia2013，，5： | 1 |
| 14 | Minocycline attenuates T cell and microglia activity to impair cytokine production in T cell-microglia interaction显示文摘 | Hader W Yong VW | 2005 | Leukoc Biol2005，78，1： | 1 |
| 15 | Relative and intermittent cardiornspiratory coordination显示文摘 | Hoyer D Hader O Zwiener U | 1997 | IEEE Eng Med Biol Mag1997，16，6： | 1 |
| 16 | Cyanobacterial biofertilizers in rice agriculture显示文摘 | VAISHAMPAYAN A SINHA R P HADER D P | 2001 | Bot Rev2001，67，： | 1 |
| 17 | Linewidth enhancement factor and optical gain in (GaIn)(Nas)/GaAs lasers显示文摘 | GERHARDT N C HOFMANN M R HADER J | 2004 | Applied Physics Letters2004，84，1： | 1 |
| 18 | Lentivirus administration to rat muscle provides efficiet sustained expression of erythropoietin显示文摘 | Seppen JG Barry SC Hader B | 2001 | Blood2001，98，3： | 1 |
| 19 | Life under solar UV radiation in aquatic organisms 显示文摘 | Sinha R P Hader D P | 2002 | Advances in Space Research2002，30，6： | 1 |
| 20 | The Erlanger flagellate test (EFT): photosynthetic flagellates in biological dosimeters显示文摘 | Hader D P Lebert M | 1997 | Photochem Photobiol1997，40，： | 1 |
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