Cappell MS, Hader I, Amin M. Acute liver failure secondary to severe systemic disease from fatal hemophagocytic lymphohistiocytosis: Case report and systematic literature review. World J Hepatol 2018; 10(9): 629-636 [PMID: 30310541 DOI: 10.4254/wjh.v10.i9.629]
Corresponding Author of This Article
Mitchell S Cappell, FACG, MD, PhD, Chief Doctor, Professor, Division of Gastroenterology and Hepatology, William Beaumont Hospital, MOB #602, 3535 W. Thirteen Mile Rd, Royal Oak, MI 48073, United States. mscappell@yahoo.com
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Systematic Reviews
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Cappell MS, Hader I, Amin M. Acute liver failure secondary to severe systemic disease from fatal hemophagocytic lymphohistiocytosis: Case report and systematic literature review. World J Hepatol 2018; 10(9): 629-636 [PMID: 30310541 DOI: 10.4254/wjh.v10.i9.629]
World J Hepatol. Sep 27, 2018; 10(9): 629-636 Published online Sep 27, 2018. doi: 10.4254/wjh.v10.i9.629
Acute liver failure secondary to severe systemic disease from fatal hemophagocytic lymphohistiocytosis: Case report and systematic literature review
Mitchell S Cappell, Ismail Hader, Mitual Amin
Mitchell S Cappell, Division of Gastroenterology and Hepatology, William Beaumont Hospital, Royal Oak, MI 48073, United States
Mitchell S Cappell, Department of Medicine, Oakland University William Beaumont School of Medicine, Royal Oak, MI 48073, United States
Ismail Hader, Department of Medicine, William Beaumont Hospital, Royal Oak, MI 48073, United States
Mitual Amin, Department of Pathology, William Beaumont Hospital, Royal Oak, MI 48073, United States
Mitual Amin, Department of Pathology, Oakland University William Beaumont School of Medicine, Royal Oak, MI 48073, United States
Author contributions: Cappell MS and Hader I are equal primary authors of this work; Hader I wrote drafts of the case report and results sections; and provided medical care for this patient; Cappell MS as mentor edited the case report section, and wrote most of the other sections; Amin M performed all the microscopic pathology, and wrote the pathologic sections of the paper.
Conflict-of-interest statement: None for all authors. In particular, Dr. Cappell, as a consultant of the United States Food and Drug Administration (FDA) Advisory Committee for Gastrointestinal Drugs, affirms that this paper does not discuss any proprietary confidential pharmaceutical data submitted to the FDA. Dr. Cappell is also a member of the speaker’s bureau for Astra Zeneca and Daiichi Sankyo, co-marketers of Movantik, and has received one-time consulting fees from Mallinckrodt and from Shire. This work does not discuss any drug manufactured or marketed by Astra Zeneca, Daiichi Sankyo, Mallinckrodt or Shire.
PRISMA 2009 Checklist statement: The authors have read the PRISMA 2009 Checklist, and the manuscript was prepared and revised according to the PRISMA 2009 Checklist.
Correspondence to: Mitchell S Cappell, FACG, MD, PhD, Chief Doctor, Professor, Division of Gastroenterology and Hepatology, William Beaumont Hospital, MOB #602, 3535 W. Thirteen Mile Rd, Royal Oak, MI 48073, United States. mscappell@yahoo.com
Telephone: +1-732-9911227 Fax: +1-248-5517581
Received: April 28, 2018 Peer-review started: May 3, 2018 First decision: May 24, 2018 Revised: July 20, 2018 Accepted: August 28, 2018 Article in press: August 28, 2018 Published online: September 27, 2018 Processing time: 152 Days and 11.4 Hours
Core Tip
Core tip: This work systematically reviews liver disease in hemophagocytic lymphohistiocytosis (HLH), proposes contraindications for liver-transplantation in such patients, and reports an illustrative case. A 47-year-old-man was hospitalized with high-grade pyrexia, total-bilirubin = 45 g/dL, and profound neutropenia. Bone marrow biopsy/aspirate revealed hemophagocytic histiocytes. The patient had HLH, satisfying 6 Histiocytic Society criteria. Liver-biopsy histopathology revealed relatively well-preserved hepatic parenchyma, except for sparse hepatocytic necrosis. The patient was not a liver-transplant-candidate, despite model for end stage liver disease-score = 33, due to multi-organ failure from HLH. He expired from multi-organ failure despite receiving corticosteroids. This case and systematic review reveals patients with HLH may not be liver transplant candidates because of liver failure from multisystem failure.
