Literature DB >> 23943807

Haemophagocytic lymphohistiocytosis presenting as liver failure following Epstein-Barr and prior hepatitis A infections.

Gineth Paola Pinto-Patarroyo1, Michael E Rytting, John Moore Vierling, Maria E Suarez-Almazor.   

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is associated with high mortality even after prompt diagnosis. We present a young man with HLH triggered by two common viral diseases, infectious mononucleosis and hepatitis A. This patient presented with fever, rapidly progressive liver failure, anasarca and cholestasis, followed by anaemia and neutropenia. His carbohydrate antigen 19-9 reached over 9000 U/mL. Initial bone marrow and liver biopsies did not show histological features of malignancy or HLH. The patient was finally diagnosed and treated almost 1 year after the initial symptoms started, and had an excellent response with etoposide and dexamethasone. This case is unusual because it was triggered following mononucleosis in a patient with positive total antibodies against hepatitis A, with rapidly developing liver failure, and also because the patient's response was excellent despite the delay in treatment. It underscores the importance of suspecting HLH when severe systemic illness develops after a viral infection, even in the absence of clear histological features.

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Year:  2013        PMID: 23943807      PMCID: PMC3761427          DOI: 10.1136/bcr-2013-008979

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  26 in total

1.  Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in adults characterized by high viral genome load within circulating natural killer cells.

Authors:  C P Fox; C Shannon-Lowe; P Gothard; B Kishore; J Neilson; N O'Connor; M Rowe
Journal:  Clin Infect Dis       Date:  2010-07-01       Impact factor: 9.079

2.  Treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH); update 2010.

Authors:  Shinsaku Imashuku
Journal:  J Pediatr Hematol Oncol       Date:  2011-01       Impact factor: 1.289

3.  HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.

Authors:  Jan-Inge Henter; Annacarin Horne; Maurizio Aricó; R Maarten Egeler; Alexandra H Filipovich; Shinsaku Imashuku; Stephan Ladisch; Ken McClain; David Webb; Jacek Winiarski; Gritta Janka
Journal:  Pediatr Blood Cancer       Date:  2007-02       Impact factor: 3.167

Review 4.  Primary and secondary hemophagocytic lymphohistiocytosis: clinical features, pathogenesis and therapy.

Authors:  Sumit Gupta; Sheila Weitzman
Journal:  Expert Rev Clin Immunol       Date:  2010-01       Impact factor: 4.473

5.  [A case of hemophagocytic syndrome complicated by acute viral hepatitis A infection].

Authors:  Ji Young Seo; Dong Dae Seo; Tae Joo Jeon; Tae Hoon Oh; Won Chang Shin; Won Choong Choi; Soo Jin Yoo; Tae Hee Han
Journal:  Korean J Hepatol       Date:  2010-03

6.  Malignancy-associated hemophagocytic lymphohistiocytosis in adults: a retrospective population-based analysis from a single center.

Authors:  Maciej Machaczka; Johan Vaktnäs; Monika Klimkowska; Hans Hägglund
Journal:  Leuk Lymphoma       Date:  2011-02-08

Review 7.  Extraordinarily elevated serum levels of CA 19-9 and rapid decrease after successful therapy: a case report and review of literature.

Authors:  Murat Korkmaz; Hakan Ünal; Haldun Selçuk; Uğur Yilmaz
Journal:  Turk J Gastroenterol       Date:  2010-12       Impact factor: 1.852

8.  The combined elevation of tumor markers CA 19-9 and CA 125 in liver disease patients is highly specific for severe liver fibrosis.

Authors:  Maximilian Schöniger-Hekele; Christian Müller
Journal:  Dig Dis Sci       Date:  2006-02       Impact factor: 3.199

9.  Hemophagocytic syndrome in a patient with acute tubulointerstitial nephritis secondary to hepatitis A virus infection.

Authors:  Eunjung Cho; Inhye Cha; Kichul Yoon; Ha Na Yang; Hye Won Kim; Myung-Gyu Kim; Sang-Kyung Jo; Won Yong Cho; Hyoung Kyu Kim
Journal:  J Korean Med Sci       Date:  2010-09-17       Impact factor: 2.153

Review 10.  Infections associated with haemophagocytic syndrome.

Authors:  Nadine G Rouphael; Naasha J Talati; Camille Vaughan; Kelly Cunningham; Roger Moreira; Carolyn Gould
Journal:  Lancet Infect Dis       Date:  2007-12       Impact factor: 25.071

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  6 in total

Review 1.  Current Updates on Classification, Diagnosis and Treatment of Hemophagocytic Lymphohistiocytosis (HLH).

Authors:  Manisha Madkaikar; Snehal Shabrish; Mukesh Desai
Journal:  Indian J Pediatr       Date:  2016-02-13       Impact factor: 5.319

Review 2.  Acute liver failure caused by hemophagocytic lymphohistiocytosis in adults: A case report and review of the literature.

Authors:  Shide Lin; Ying Li; Jun Long; Qichuan Liu; Fangwan Yang; Yihuai He
Journal:  Medicine (Baltimore)       Date:  2016-11       Impact factor: 1.889

3.  Haemophagocytic lymphohistiocytosis associated with fulminant hepatitis and multiorgan failure following primary Epstein-Barr virus and herpes simplex virus type 1 infection.

Authors:  Claudia Honsig; Sandra Beinhardt; Josef Tomasits; Hans Peter Dienes
Journal:  BMJ Case Rep       Date:  2017-03-29

4.  Acute liver failure secondary to severe systemic disease from fatal hemophagocytic lymphohistiocytosis: Case report and systematic literature review.

Authors:  Mitchell S Cappell; Ismail Hader; Mitual Amin
Journal:  World J Hepatol       Date:  2018-09-27

5.  Reactivation of Epstein-Barr Virus Hepatitis in T/Natural Killer (NK) Cells Mimicking Liver T/NK-Cell Lymphoma.

Authors:  Sara Kwong; Xiangping Lu; Xiuli Liu; Jinping Lai
Journal:  Gastroenterology Res       Date:  2020-04-22

6.  Acute liver injury secondary to hemophagocytic lymphohistiocytosis triggered by Epstein-Barr virus infection.

Authors:  Aiji Hattori; Yasuhiko Hamada; Hiroyuki Kawabata; Kyosuke Tanaka
Journal:  JGH Open       Date:  2020-10-22
  6 in total

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