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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 | Peripartum angiopathy with simultaneous sinus venous thrombosis, cervical artery dissection and cerebral arterial vasoconstriction显示文摘 | Hoeren M Hader C Strumpell S | 2011 | J Neurol2011，258，11： | 1 |
| 3 | Muhichip vertical-externalcavity surface-emitting lasers:a coherent power scaling scheme显示文摘 | Fan L Fallahi M Hader J Zakharian A R Moloney J V Murray J T Bedford R Stolz W Koch S W | | 0，，24： | 1 |
| 4 | Extended tunability in a two-chip VECSEL显示文摘 | Fan L Fallahi M Zakharian A Hader J Moloney J V Bedford R Murray J T Stolz W Koch S W | | 0，，08： | 1 |
| 5 | 5-W yellow laser by intracavity frequency doubling of high-power verticalexternal-cavity surface-emitting laser显示文摘 | Fallahi M Fan L Kaneda Y Hessenius C Hader J Li H Moloney J V Kunert B Stolz W Koch S W Murray J Bedford R | | 0，，20： | 1 |
| 6 | Tunable high-power high-brightness linearly polarized vertical-external-cavity surfaceemitting lasers显示文摘 | Fan L Fallahi M Murray J T Bedford R Kaneda Y Zakharian A R Hader J Moloney J V Stolz W Koch S W | | 0，，02： | 1 |
| 7 | Cloning and expression of Drosophila SOCS36E and its potential regulation by the JAK/STAT pathway 显示文摘 | Karsten P Hader S Zeidler M P | 2002 | Mech Dev2002，117，12： | 1 |
| 8 | Role of lipids and fatty acids in stress tolerance in Cyanobaeteria 显示文摘 | SINGH S C SINHA R P HADER D P | 2002 | Acta Protozool2002，41，4： | 1 |
| 9 | Effects of solar radiation on motility and pigmentation of three species of phytoplankton 显示文摘 | Gerber S Hader D P | 1993 | Environmental and Experimental Botany1993，33，4： | 1 |
| 10 | Density-activated defect recombination as a possible explanation for the efficiency droop in Ga N-based diodes显示文摘 | HADER J MOLONEY J V KOCH S W | 2010 | Applied Physics Letters2010，96，22： | 1 |
| 11 | Detection of reac- tive oxygen species (ROS) by the oxidant-sensing probe 2', 7'-dichlorodihydrofluorescein diacetate in the cyanobacte- rium Anabaena variabilis PCC 7937 显示文摘 | Rastogi R P Singh S P Hader D P | 2010 | Biochemical and Biophysical Research Communications2010，397，3： | 1 |
| 12 | Role of lipids and fatty acids in stress tolerance in Cyanobacteria 显示文摘 | Singh S C Sinha R P Hader D P | 2002 | Acta Protozool2002，41，： | 1 |
| 13 | Directly administered antiretroviral therapy in an urban methadone maintenance clinic: a nonrandanized comparative study显示文摘 | Lucas GM Weidle P J Hader S | 2004 | Clin Infect Dis2004，38，5： | 1 |
| 14 | Quantum design of semiconductor active materials laser and amplifier applications 显示文摘 | MOLONEY J V HADER J KOCH S W | 2007 | Laser & Photon Rev2007，1，： | 1 |
| 15 | Chemistry of reclaimed rubber显示文摘 | Hader R N LeBeau D S | 1951 | Industrial and Engineering Chemistry1951，，43： | 1 |
| 16 | Detection of re- active oxygen species (ROS) by the oxidant-sensing probe 2' , 7' -dichlorodihydrofluorescein diacetate in the cyanobacteriumAnabaena variabilis PCC 7937显示文摘 | Rastogi R P Singh S P Hader D | 2010 | Bio- chem Biophys Res Communic2010，397，3： | 1 |
| 17 | Directly administered antiretroviral therapy in an urban methadone maintenance clinic a onrandomized comparative study 显示文摘 | LUCAS G M WEIDE P J HADER S | 2004 | Clin Infect Dis2004，38，5： | 1 |
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