Natural Killer–Like T-Cell Lymphoma: A Rare Cause of Acute Liver Failure

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ABSTRACT

Acute liver failure is characterized by encephalopathy and disruption of hepatic function, often requiring liver transplantation to prevent fatal consequences. We present a 33-year-old man with recurrent lymphoma presenting with acute liver failure, which was initially thought to be from drug-induced liver injury associated with his chemotherapy medication, asparaginase. However, liver biopsy revealed malignant infiltration by lymphoma. The subtype of lymphoma was natural killer–like T-cell lymphoma, which is an uncommon variant, and has rarely been associated with hepatic infiltration. His condition rapidly worsened with development of multiorgan failure leading to death.

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Acute liver failure: Summary of a workshop.

Edward Doo, L Nyberg, Charles Squires … (2008)

Acute liver failure (ALF) is a rare but challenging clinical syndrome with multiple causes; a specific etiology cannot be identified in 15% of adult and 50% of pediatric cases. The course of ALF is variable and the mortality rate is high. Liver transplantation is the only therapy of proven benefit, but the rapidity of progression and the variable course of ALF limit its use. Currently in the United States, spontaneous survival occurs in approximately 45%, liver transplantation in 25%, and death without transplantation in 30% of adults with ALF. Higher rates of spontaneous recovery (56%) and transplantation (31%) with lower rates of death (13%) occur in children. The outcome of ALF varies by etiology, favorable prognoses being found with acetaminophen overdose, hepatitis A, and ischemia (approximately 60% spontaneous survival), and poor prognoses with drug-induced ALF, hepatitis B, and indeterminate cases (approximately 25% spontaneous survival). Excellent intensive care is critical in management of patients with ALF. Nonspecific therapies are of unproven benefit. Future possible therapeutic approaches include N-acetylcysteine, hypothermia, liver assist devices, and hepatocyte transplantation. Advances in stem cell research may allow provision of cells for bioartificial liver support. ALF presents many challenging opportunities in both clinical and basic research.

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NKT-cell subsets: promoters and protectors in inflammatory liver disease.

Vipin Kumar (2013)

Natural killer T cells (NKT) are innate-like cells which are abundant in liver sinusoids and express the cell surface receptors of NK cells (e.g., NK1.1 (mouse) or CD161+/CD56+(human)) as well as an antigen receptor (TCR) characteristic of conventional T cells. NKT cells recognize lipid antigens in the context of CD1d, a non-polymorphic MHC class I-like molecule. Activation of NKT cells has a profound influence on the immune response against tumors and infectious organisms and in autoimmune diseases. NKT cells can be categorized into at least two distinct subsets: iNKT or type I use a semi-invariant TCR, whereas type II NKT TCRs are more diverse. Recent evidence suggests that NKT-cell subsets can play opposing roles early in non-microbial liver inflammation in that type I NKT are proinflammatory whereas type II NKT cells inhibit type I NKT-mediated liver injury.

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Acute liver failure secondary to hepatic infiltration: a single centre experience of 18 cases.

Ashley R. Williams, J Wendon, D S Rowbotham (1998)

Acute liver failure (ALF) secondary to malignant infiltration of the liver is rare and is diagnosed often only after death. To determine diagnostic factors and particular clinical patterns of illness. Review of case notes from all patients with ALF secondary to hepatic infiltration admitted to this unit over an 18 year period (1978-1995). From a total of 4020 admissions, 18 patients were identified with ALF attributable to hepatic infiltration. Mean age was 40.7 years. Aetiology was non-Hodgkin's lymphoma in nine patients, Hodgkin's disease in three, infiltrative metastatic carcinoma in four, and haemophagocytosis with no precipitant cause in two cases. Prodromal symptoms were non-specific, but occurred at least two to four weeks before onset of ALF, making the presence of such symptoms of value in differential diagnosis of the cause of ALF. Clinical examination and investigations were unhelpful in distinguishing these cases from more usual causes of ALF. Usually, the clinical course was of rapid deterioration and death from multiorgan failure, and only one patient survived. Diagnosis was made during life in 15 patients. Histology showed evidence of widespread hepatocellular necrosis, with diffuse infiltration by tumour cells rather than focal cellular aggregation. Only with accurate histological diagnosis from liver biopsy and institution of specific therapy early in the management of such patients will the best chance of recovery be achieved. In every case of ALF with prodromal symptoms or abnormal imaging, hepatic histology should be obtained by liver biopsy as soon as possible to diagnose infiltrative hepatic disease.

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Journal

Journal ID (nlm-ta): ACG Case Rep J

Journal ID (iso-abbrev): ACG Case Rep J

Journal ID (coden): ACGCRJ

Journal ID (pmc): ACGCRJ

Journal ID (publisher-id): AC9

Title: ACG Case Reports Journal

Publisher: Wolters Kluwer (Maryland, MD )

ISSN (Electronic): 2326-3253

Publication date (Electronic): 17 July 2019

Publication date Collection: July 2019

Volume: 6

Issue: 7

Electronic Location Identifier: e00145

Affiliations

[1 ]Department of Internal Medicine, St. Joseph's Hospital and Medical Center, Phoenix, AZ

[2 ]Department of Gastroenterology, St. Joseph's Hospital and Medical Center, Phoenix, AZ

[3 ]Department of Transplant Hepatology, St. Joseph's Hospital and Medical Center, Phoenix, AZ

Author notes

Correspondence: Ishani Shah, MD, Department of Internal Medicine, St. Joseph's Hospital and Medical Center, 350 W Thomas Rd, Phoenix, AZ 85013 ( Ishani.Shah@ 123456dignityhealth.org ).

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Publisher ID: ACGCR-19-0019 Accession ID: 00017

DOI: 10.14309/crj.0000000000000145

PMC ID: 6722367

SO-VID: ac9468c9-1593-4885-94f6-8b14fc4b3f6f

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This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

History

Date received : 24 January 2019

Date accepted : 31 May 2019

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Natural Killer–Like T-Cell Lymphoma: A Rare Cause of Acute Liver Failure

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Karger: Oncology

Most cited references 19

Acute liver failure: Summary of a workshop.

NKT-cell subsets: promoters and protectors in inflammatory liver disease.

Acute liver failure secondary to hepatic infiltration: a single centre experience of 18 cases.

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