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1
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Yao J, Yan Y, Ruan M, Lin H, Li D, Zeng Z. Drug-induced Autoimmune-like Hepatitis With Pathological Features of Giant Cell Hepatitis. J Clin Exp Hepatol 2025; 15:102498. [PMID: 39917420 PMCID: PMC11795586 DOI: 10.1016/j.jceh.2024.102498] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/26/2024] [Accepted: 12/24/2024] [Indexed: 02/09/2025] Open
Abstract
Background and methods The differentiation between drug-induced autoimmune-like hepatitis (DI-ALH) and autoimmune hepatitis (AIH) can be confusing and challenging. We present a case of DI-ALH characterized by positive autoantibodies, elevated immunoglobulin G (IgG) levels and histology characteristics of giant cell hepatitis. Results A liver biopsy was performed for etiology clarification, DI-ALH was diagnosed. Following treatment with corticosteroids and azathioprine, the patient's liver function remained normal without recurrence. Conclusions Distinguishing DI-ALH from AIH based on the possibility of discontinuing immunosuppression and the histological features. Clinicians need to investigate patient's medical history, serological tests, and pathological changes to establish a correct diagnosis.
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Affiliation(s)
- Jie Yao
- Department of Hepatobiliary Disease, Fuzhou General Clinical Medical College of Fujian Medical University, The 900th Hospital of Joint Logistics Support Force, No. 156 West Second Ring North Road, Gulou District, Fuzhou, Fujian, 350025, China
- Department of Hepatobiliary Disease, The 900th Hospital of Joint Logistics Support Force, No. 156 West Second Ring North Road, Gulou District, Fuzhou, Fujian, 350025, China
| | - Yongqin Yan
- Department of Pathology, The 900th Hospital of Joint Logistics Support Force, No. 156 West Second Ring North Road, Gulou District, Fuzhou, Fujian, 350025, China
| | - Mei Ruan
- Department of Hepatobiliary Disease, The Third People's Hospital of Fujian University of Traditional Chinese Medicine, No. 363 Guobin Avenue, Minhou Street, Fuzhou, Fujian, 350100, China
| | - Haiyan Lin
- Department of Hepatobiliary Disease, The 900th Hospital of Joint Logistics Support Force, No. 156 West Second Ring North Road, Gulou District, Fuzhou, Fujian, 350025, China
| | - Dongliang Li
- Department of Hepatobiliary Disease, Fuzhou General Clinical Medical College of Fujian Medical University, The 900th Hospital of Joint Logistics Support Force, No. 156 West Second Ring North Road, Gulou District, Fuzhou, Fujian, 350025, China
- Department of Hepatobiliary Disease, The 900th Hospital of Joint Logistics Support Force, No. 156 West Second Ring North Road, Gulou District, Fuzhou, Fujian, 350025, China
| | - Zhiyu Zeng
- Department of Hepatobiliary Disease, The 900th Hospital of Joint Logistics Support Force, No. 156 West Second Ring North Road, Gulou District, Fuzhou, Fujian, 350025, China
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2
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Teker Düztaş D, Öztürk H, Eğritaş Gürkan Ö, Esendağlı G, Sarı S, Dalgıç B, Dalgıç A. Overview of the Etiology, Pathology, and Prognosis of Giant Cell Hepatitis. EXP CLIN TRANSPLANT 2024; 22:106-110. [PMID: 39498930 DOI: 10.6002/ect.pedsymp2024.o30] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/07/2024]
Abstract
OBJECTIVES Giant cell hepatitis is an important diagnostic consideration in early childhood, especially for patients who present with jaundice. Different diseases may play a role in their etiology. In this study, we presented pediatric patients in our center diagnosed with giant cell hepatitis. MATERIALS AND METHODS Liver biopsies performed in our hospital between January 2010 and December 2023 were retrospectively evaluated, and biopsies with histological giant cell formation were included in the study. Demographic, clinical, and histopathological characteristics of the patients were obtained from the medical records. RESULTS Our study included 31 cases of giant cell hepatitis. Twenty-four (90.3%) of the patients were male, and the median age was 4 months (range, 1-148 mo). The most common clinical findings were jaundice (93.5%). Among the 31 patients, idiopathic giant cell hepatitis was observed in 13 cases (41.9%), and etiology was elucidated in 18 patients. The most common diagnoses in these patients were progressive familial intrahepatic cholestasis, biliary atresia, and autoimmune hepatitis (22.6%, 19.4%, and 9.7%, respectively). The most common accompanying histopathologic findings were canalicular cholestasis (87.1%), lobular cholestasis (54.8%), and pericellular fibrosis (35.5%). Liver transplant was required in 10 patients (32.3%). Nine patients (29%) died from various reasons. Although follow-up of 13 patients (41.9%) are ongoing, 9 patients (29%) are no longer being followed. CONCLUSIONS Few studies in the pediatric age group on giant cell hepatitis have been conducted in the literature. Similar to our series, etiology could not be specified in most of the patients in these reports. Additional research is needed to improve our understanding of the disease process, uncover any other potential causes, and create specific treatment options.
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Affiliation(s)
- Demet Teker Düztaş
- From the Department of Pediatric Gastroenterology, Gazi University School of Medicine, Ankara, Turkey
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Jiao J, Zhang X. Post-infantile Giant Cell Hepatitis: A Literature Review and Meta-analysis. JOURNAL OF CLINICAL AND TRANSLATIONAL PATHOLOGY 2022; 2:100-107. [PMID: 37092012 PMCID: PMC10117396 DOI: 10.14218/jctp.2022.00016] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
Post-infantile giant cell hepatitis (PIGCH) is a rare disease entity in adults with a multifactorial etiology and widely variable clinical courses and outcomes. The factors associated with the worse outcomes of this disease entity are still unclear. We identified 68 PIGCH patients by searching PubMed and performed meta-analysis. Among the 68 patients, 32% of the cases were associated with autoimmune disorders, followed by 21% associated with viral infections, 10% with medication, and 7% with malignancy. Twenty-four percent of the patients had more than one etiological factor, and 6% had other uncommon etiologies or an etiology that could not be identified. At the time of this report, 17 patients had died of the disease (poor outcome), and 51 patients remained alive with the disease (good outcome). Compared to the patients with a good outcome, the patients with a poor outcome were characterized by older age, lower levels of platelets and albumin, higher level of total bilirubin, and a diffuse distribution pattern of giant cells in the liver. There were no differences in gender distribution, aminotransferase, alkaline phosphatase, gamma-glutamyl transferase, etiological distribution, or other histological features, including interface hepatitis, necrosis, lobular inflammation, portal inflammation, cholestasis, or fibrosis. Further studies would be needed to better understand the disease mechanisms and unmask any additional etiological factors and targeted therapies.
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Affiliation(s)
| | - Xuchen Zhang
- Correspondence to: Xuchen Zhang, Department of Pathology, Yale University School of Medicine, 310 Cedar Street, PO Box 208023, New Haven, CT 06510, United States. Tel: +1 203-785-6010, Fax: +1 203-737-2922,
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4
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Bonilla Gonzalez C, Hincapié Echeverría M, Plazas Pachón R, Mora Umaña P, Diaz Gómez BL, Gualdron Barreto N. Case Report: Fatal Acute Liver Failure With Giant Cell Transformation in a Pediatric Patient Associated With MIS-C. Front Pediatr 2022; 9:780258. [PMID: 35127589 PMCID: PMC8815702 DOI: 10.3389/fped.2021.780258] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/20/2021] [Accepted: 11/22/2021] [Indexed: 12/15/2022] Open
Abstract
Background: We describe the first pediatric case of a 10-month-old boy with MIS-C who developed fulminant acute liver failure with associated giant cell transformation and a fatal outcome, after ruling out other infectious, metabolic, genetic, and autoimmune causes of liver failure following the usual algorithms for approaching the etiology. Although the patient received the main treatment strategies for liver failure, he had a fatal outcome. A clinical autopsy was considered as part of the diagnostic approach, which showed evidence of giant cell transformation.
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Affiliation(s)
- Carolina Bonilla Gonzalez
- Pediatric Intensive Care Unit, Department of Pediatrics, Fundación Santafe of Bogotá, Bogotá, Colombia
| | | | - Rocio Plazas Pachón
- Pediatric Residents Andes University, Fundación Santafe of Bogotá, Bogotá, Colombia
| | - Paola Mora Umaña
- Pediatric Residents Andes University, Fundación Santafe of Bogotá, Bogotá, Colombia
| | | | - Nathalie Gualdron Barreto
- Pediatric Intensive Care Unit, Department of Pediatrics, Fundación Santafe of Bogotá, Bogotá, Colombia
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5
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Tan YW, Wang JM, Chen L. Is simultaneous presence of IgG4-positive plasma cells and giant-cell hepatitis a coincidence in autoimmune hepatitis? A case report. World J Clin Cases 2021; 9:7527-7534. [PMID: 34616822 PMCID: PMC8464467 DOI: 10.12998/wjcc.v9.i25.7527] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/18/2021] [Revised: 04/24/2021] [Accepted: 07/19/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND The immune-mediated invasion of IgG4-positive plasma cells in the liver is found in some autoimmune hepatitis. Giant-cell hepatitis (GCH) is a very rare pathological feature in adults, and the clinical characteristics of the simultaneous appearance of the two pathological phenomena are not clear.
CASE SUMMARY A 68-year-old woman was hospitalized with fatigue, poor appetite, and yellow urine for 20 d. Liver function tests and immunological indexes were significantly abnormal and accompanied by elevated serum IgG4 levels. Liver pathology revealed severe inflammation of the interface between the portal tract and hepatocytes, portal area inflammation, plasma cell infiltration, formation of rosette cells, IgG4-positive plasma cells > 10/high-power field, IgG4/IgG > 40%, and multinucleated liver cell swelling. IgG4-related autoimmune hepatitis (AIH) combined with GCH was diagnosed, and methylprednisolone was administered at 40 mg/day. Two weeks later, the clinical symptoms disappeared, and the liver function and immunological indicators were significantly improved. Methylprednisolone was reduced at a rate of 4–8 mg per week to 8 mg/day for maintenance. A second liver biopsy 48 wk later indicated that liver inflammation and fibrosis were significantly improved. IgG4-positive plasma cells and GCH were not detected. A literature search was conducted to analyze articles reporting similar pathological phenomena.
CONCLUSION AIH with simultaneous IgG4-positive plasma cell infiltration and GCH, liver inflammation, and fibrosis is possibly more severe than typical AIH but sensitive to corticosteroids.
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Affiliation(s)
- You-Wen Tan
- Department of Hepatology, The Third Hospital of Zhenjiang Affiliated Jiangsu University, Zhenjiang 212003, Jiangsu Province, China
| | - Jia-Min Wang
- Department of Hepatology, The Third Hospital of Zhenjiang Affiliated Jiangsu University, Zhenjiang 212003, Jiangsu Province, China
| | - Li Chen
- Department of Hepatology, The Third Hospital of Zhenjiang Affiliated Jiangsu University, Zhenjiang 212003, Jiangsu Province, China
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6
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Covelli C, Sacchi D, Sarcognato S, Cazzagon N, Grillo F, Baciorri F, Fanni D, Cacciatore M, Maffeis V, Guido M. Pathology of autoimmune hepatitis. Pathologica 2021; 113:185-193. [PMID: 34294936 PMCID: PMC8299324 DOI: 10.32074/1591-951x-241] [Citation(s) in RCA: 31] [Impact Index Per Article: 7.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/14/2020] [Accepted: 12/14/2020] [Indexed: 12/12/2022] Open
Abstract
Autoimmune hepatitis (AIH) is a relatively rare non-resolving chronic liver disease, which mainly affects women. It is characterized by hypergammaglobulinemia, circulating autoantibodies, interface hepatitis on liver histology and a favourable response to immunosuppression. The putative mechanism for the development of autoimmune hepatitis is thought to be the interaction between genetic predisposition, environmental triggers and failure of the native immune system. AIH still remains a major diagnostic and therapeutic challenge, mainly because it is a very heterogeneous disease. Prompt and timely diagnosis is crucial since, if left untreated, AIH has a high mortality rate. Histological demonstration of hepatitis is required for the diagnosis of AIH and, therefore, liver biopsy is mandatory in the initial diagnostic work-up, before treatment. In this review, we summarize the histological features of AIH with the main aim of highlighting the most important clinical-pathological hallmarks useful in the routine diagnostic practice.
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Affiliation(s)
- Claudia Covelli
- Pathology Unit, Fondazione IRCCS Casa Sollievo della Sofferenza, San Giovanni Rotondo, Italy
| | - Diana Sacchi
- Pathology Department Azienda ULSS 2 Marca Trevigiana, Treviso, Italy
| | | | - Nora Cazzagon
- Department of Surgery, Oncology and Gastroenterology, University of Padova, Padova, Italy
| | - Federica Grillo
- Unit of Anatomic Pathology, Department of Surgical Sciences and Integrated Diagnostics (DISC), University of Genova and Ospedale Policlinico San Martino IRCCS, Genoa, Italy
| | | | - Daniela Fanni
- Pathology Unit, Department of Medical Sciences, University of Cagliari, Cagliari, Italy
| | | | - Valeria Maffeis
- Pathology Department Azienda ULSS 2 Marca Trevigiana, Treviso, Italy
| | - Maria Guido
- Pathology Department Azienda ULSS 2 Marca Trevigiana, Treviso, Italy.,Department of Medicine-DIMED, University of Padova, Padova, Italy
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7
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Xiao Y, Gao ZH, Deschenes M. Autoimmune post-infantile giant cell hepatitis: a case report and review of the literature. CANADIAN LIVER JOURNAL 2021; 4:104-109. [PMID: 35991767 PMCID: PMC9204937 DOI: 10.3138/canlivj-2020-0024] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/05/2020] [Accepted: 09/27/2020] [Indexed: 08/08/2023]
Abstract
Giant cell hepatitis (GCH) is a rare entity in adults that is characterized by large multinucleated hepatocyte formation and parenchymal inflammation. We present a case of acute liver failure in a 33-year-old woman secondary to autoimmune hepatitis (AIH). A liver biopsy revealed submassive hepatocyte necrosis consistent with GCH. We conducted a literature review of 187 reported cases of post-infantile GCH in adults. AIH was the most commonly reported cause of GCH, but GCH was associated with a wide spectrum of etiologies, including infections, rheumatological diseases, hematological diseases, malignancies, and medications. The severity of disease can range from mild hepatitis to fulminant hepatic failure. The mortality rate among the cases in the literature was 18.82%. GCH is managed by treating the underlying cause, and ribavirin has been proposed as a treatment option for idiopathic GCH. A small number of patients progress to requiring orthotopic liver transplant, but recurrence is possible post-transplant.
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Affiliation(s)
- Yasi Xiao
- Department of Internal Medicine, McGill University, Montreal, Quebec, Canada
| | - Zu-Hua Gao
- Department of Pathology, McGill University, Montreal, Quebec, Canada
| | - Marc Deschenes
- Division of Gastroenterology, McGill University, Montreal, Quebec, Canada
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8
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Poddighe D, Madiyeva A, Talipova D, Umirbekova B. Infantile giant cell hepatitis with autoimmune hemolytic anemia. World J Hepatol 2021; 13:411-420. [PMID: 33959224 PMCID: PMC8080548 DOI: 10.4254/wjh.v13.i4.411] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/09/2021] [Revised: 01/26/2021] [Accepted: 03/08/2021] [Indexed: 02/06/2023] Open
Abstract
Giant cell hepatitis (GCH) is characterized by large and multinucleated (syncytial) hepatocytes in the context of liver inflammation. Infantile GCH is typically associated with autoimmune hemolytic anemia in the absence of any other systemic or organ-specific autoimmune comorbidity. The etiology is unknown; concomitant viral infections (as potential trigger factors) have been identified in a few patients. The pathogenesis reportedly relies upon immune-mediated/ autoimmune mechanisms. This condition should be considered in any infant developing Coombs-positive anemia; indeed, anemia usually precedes the development of hepatitis. The clinical course is usually aggressive without the appropriate immunosuppressive therapy, which may include steroids, conventional immunosuppressors (e.g., azathioprine and cyclophosphamide as first-line treatments), intravenous immunoglobulin, and biologics (rituximab). Improvements in medical management (including the availability of rituximab) have significantly reduced the mortality of this condition in the last decade.
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Affiliation(s)
- Dimitri Poddighe
- Department of Medicine, Nazarbayev University School of Medicine, Nur-Sultan 010000, Kazakhstan
- Department of Pediatrics, National Research Center for Maternal and Child Health, Nur-Sultan 010000, Kazakhstan.
| | - Aidana Madiyeva
- Department of Medicine, Nazarbayev University School of Medicine, Nur-Sultan 010000, Kazakhstan
| | - Diana Talipova
- Department of Medicine, Nazarbayev University School of Medicine, Nur-Sultan 010000, Kazakhstan
| | - Balzhan Umirbekova
- Department of Pediatrics, National Research Center for Maternal and Child Health, Nur-Sultan 010000, Kazakhstan
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9
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Rauf M, Sen S, Levene A, Nisar MK. Giant Cell Hepatitis - A Rare Association with Connective Tissue Disease. Mediterr J Rheumatol 2020; 30:224-227. [PMID: 32467874 PMCID: PMC7241662 DOI: 10.31138/mjr.30.4.224] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/25/2019] [Revised: 11/09/2019] [Accepted: 11/11/2019] [Indexed: 11/04/2022] Open
Abstract
A 68-year-old gentleman presented to hepatology department with asymptomatic year-long history of stably deranged liver function tests. His peak alkaline phosphatase (ALP), was 828 with alanine transaminase (ALT) of 141. Full liver workup was negative; hence, a liver biopsy was organised, which confirmed giant cell hepatitis (GCH). A computed tomography (CT) scan revealed non-specific interstitial pneumonitis (NSIP) pattern interstitial lung disease supported by lung function tests. Antibody testing showed strongly positive antinuclear antibody (ANA) with anti-polymyositis/scleroderma (anti-PM-SCL) antibody. Clinical picture was in keeping with likely undifferentiated connective tissue disease (UCTD) with polyarthralgia, early morning stiffness, Raynaud's and nailfold infarcts with capillaritis on nail bed examination. Further testing confirmed triple-positive antiphospholipid antibodies twice 12 weeks apart (immunoglobulin M [IgM] anti beta-2 glycoprotein antibodies, lupus anticoagulant and IgM anticardiolipin antibody). He was treated with mycophenolate and hydroxychloroquine with resolution of symptoms. Giant cell hepatitis is uncommon, with only 100 cases reported worldwide. To our knowledge, this is the only report of GCH in the context of UCTD, highlighting the significance of careful evaluation of liver disease overlap and the successful role of mycophenolate mofetil (MMF) in this setting.
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Affiliation(s)
- Maria Rauf
- Histopathology Department, Luton & Dunstable University Hospital, Luton, United Kingdom
| | - Sambit Sen
- Hepatology Department, Luton & Dunstable University Hospital, Luton, United Kingdom
| | - Adam Levene
- Histopathology Department, Luton & Dunstable University Hospital, Luton, United Kingdom
| | - Muhammad K Nisar
- Rheumatology Department, Luton & Dunstable University Hospital, Luton, United Kingdom
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10
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Matta B, Cabello R, Rabinovitz M, Minervini M, Malik S. Post-infantile giant cell hepatitis: A single center’s experience over 25 years. World J Hepatol 2019; 11:752-760. [PMID: 31966907 PMCID: PMC6960295 DOI: 10.4254/wjh.v11.i12.752] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/14/2019] [Revised: 09/06/2019] [Accepted: 10/19/2019] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Giant cell hepatitis in the adult population remains very poorly defined with only 100 case reports published in the literature over the last three decades.
AIM To present our center’s experience in an attempt to learn about the predisposing factors, outcomes and efficacy of proposed therapeutic interventions for giant cell hepatitis.
METHODS A retrospective chart review was conducted through the electronic records of the University of Pittsburgh Medical Center. We queried 36726 liver biopsy reports from January 1, 1991 to December 6, 2016. Our search yielded 50 patients who were identified as carrying a definite diagnosis of post-infantile giant cell hepatitis (PIGCH) by pathology. The data collected included demographic information, laboratory data (liver function tests, autoimmune markers) and transplant status. In order to better analyze patient characteristics and outcomes, subjects were separated into a non-transplant (native) liver group and a post-liver transplant (allograft) group.
RESULTS The incidence of PIGCH was approximately 0.14% of all biopsies queried in the 25-year period. The mean age was 48 years with 66% females. Liver function tests were classified as 38.2% cholestatic, 35.3% hepatocellular and 26.5% mixed. Autoimmune hepatitis was found to be the most prevalent predisposing factor leading to PIGCH constituting 32% of cases. Management consisted mainly of immunosuppression, viral targeted therapy, supportive care and in six cases liver transplantations.
CONCLUSION The diagnosis of PIGCH remains clinically challenging and requires a high index of suspicion as well as a thorough history, physical examination, serological workup and liver biopsy. Treatment of the underlying cause can result in clinical stability in a large number of cases.
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Affiliation(s)
- Bassem Matta
- Department of Medicine, Division of Gastroenterology, Hepatology and Nutrition, University of Pittsburgh, Pittsburgh, PA 15213, United States
| | - Ricardo Cabello
- Department of Internal Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA 15213, United States
| | - Mordechai Rabinovitz
- Department of Medicine, Division of Gastroenterology, Hepatology and Nutrition, University of Pittsburgh, Pittsburgh, PA 15213, United States
| | - Marta Minervini
- Department of Pathology, University of Pittsburgh, Pittsburgh, PA 15213, United States
| | - Shahid Malik
- Department of Medicine, University of Pittsburgh, Division of Gastroenterology Hepatology and Nutrition, Pittsburgh, PA 15213, United States
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Sergienkci EN, Romanova ON, Klyuchareva AA, Klimovich NV, Kletskiy SK, Sakharov IV, Lisitskaya TI, Kashkan AM, Ocheretny MD, Buldyk EA, Grinchak VP, Strizhak YV. Clinical observations of giant cell hepatitis in children. JOURNAL INFECTOLOGY 2019; 11:153-160. [DOI: 10.22625/2072-6732-2019-11-4-153-160] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/06/2025]
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12
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Kethireddy N, Boyle E, Haley M, Reddy A, Forouhar F, Clement J. CLL associated giant cell hepatitis. Leuk Res 2019; 82:43-45. [PMID: 31170661 DOI: 10.1016/j.leukres.2019.05.011] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/18/2019] [Revised: 05/22/2019] [Accepted: 05/23/2019] [Indexed: 10/26/2022]
Affiliation(s)
| | - Evan Boyle
- University of Connecticut, United States
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13
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Asymptomatic giant cell hepatitis: a subtype of post-infantile giant cell hepatitis? Clin J Gastroenterol 2019; 12:367-371. [PMID: 30767175 DOI: 10.1007/s12328-019-00950-6] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/20/2018] [Accepted: 02/09/2019] [Indexed: 01/23/2023]
Abstract
Giant cell hepatitis in adults is considered a rapidly progressive and life-threatening disease, but there are few descriptions of a prolonged disease course. A 36-year-old Japanese man was referred to our hospital for further evaluation of abnormal liver function test results. Although asymptomatic, he had undergone follow-up for 9 years with these abnormalities. Because the cause of liver injury was not identified despite extensive noninvasive examinations, the patient underwent needle biopsy. He was finally diagnosed with post-infantile giant cell hepatitis (PIGCH) based on the presence of small numbers of giant multinucleated hepatocytes scattered primarily around the portal area. Necroinflammatory changes were very mild in the portal tracts and hepatic parenchyma. According to the histological findings as well as the accepted international diagnostic scoring system for autoimmune hepatitis (AIH), which is closely related to PIGCH, AIH was unlikely, although antinuclear antibody was positive at a titer of 1:160. The present case may describe an unknown subtype of PIGCH, characterized by insidious disease onset and progression with concurrent, mildly active underlying hepatitis, which is in contrast with the well-documented aggressive nature of PIGCH.
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14
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Postinfantile Giant Cell Hepatitis with Features of Acute Severe Autoimmune Hepatitis Probably Triggered by Diclofenac in a Patient with Primary Myelofibrosis. Case Reports Hepatol 2018; 2018:9793868. [PMID: 29713554 PMCID: PMC5866856 DOI: 10.1155/2018/9793868] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/10/2017] [Revised: 01/11/2018] [Accepted: 02/11/2018] [Indexed: 01/28/2023] Open
Abstract
Giant cell hepatitis (GCH) is commonly reported in neonatal and infantile liver diseases but rarely in adults where the term postinfantile GCH (PIGCH) is used. PIGCH is associated with many diseases, including drugs toxicity, viruses, and autoimmune liver diseases, with autoimmune hepatitis (AIH) being the most prevalent. We present a case of PIGCH in a 76-year-old female without known history of liver disease who suffered from an acute severe episode of hepatitis. After careful exclusion of other hepatitis causes by imaging, virological, immunological, and microbiological investigations, a diagnosis of acute severe AIH (AS-AIH) was established. The patient was started on corticosteroids but she did not respond and died 3 days later because of advanced acute liver failure. Postmortem liver biopsy showed typical PIGCH lesions. Physicians must keep this catastrophic entity in mind in cases of unexplained acute liver injury as, contrary to our case, prompt rescue therapy with corticosteroids may be life-saving.
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15
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Shetty S, Janarthanan K, Leelakrishnan V, Nirmala V. Giant-cell Hepatitis-Rare Entity in Adults. J Clin Exp Hepatol 2016; 6:244-245. [PMID: 27746622 PMCID: PMC5052396 DOI: 10.1016/j.jceh.2016.02.007] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/10/2015] [Accepted: 02/25/2016] [Indexed: 12/12/2022] Open
Abstract
Giant-cell hepatitis (GCH) is characterized by parenchymal inflammation with formation of large multinucleated hepatocytes in response to a variety of insults to the liver. Although it is commonly described in neonates, it rarely occurs in adults. Here we report a case of GCH because of herbal medicine intake.
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Affiliation(s)
- Shiran Shetty
- Department of Gastroenterology and Hepatology, Kasturba Medical College, Manipal University, Manipal 576 104, India,Address for correspondence: Shiran Shetty, Associate Professor, Department of Gastroenterology and Hepatology, Kasturba Medical College, Manipal University, Manipal 576 104, Karnataka, India. Tel.: +91 820 2922192; fax: +91 820 2571934.Shiran Shetty, Associate Professor, Department of Gastroenterology and Hepatology, Kasturba Medical College, Manipal UniversityManipal576 104India
| | - Krishnaveni Janarthanan
- Department of Gastroenterology and Hepatology, Kasturba Medical College, Manipal University, Manipal 576 104, India,PSG Institute of Medical Sciences and Research, Coimbatore 641004, Tamilnadu, India
| | | | - Vadakkepat Nirmala
- PSG Institute of Medical Sciences and Research, Coimbatore 641004, Tamilnadu, India
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Abstract
While it commonly occurs in the pediatric population, syncytial giant cell hepatitis is rare in adults, which is diagnosed histologically by the presence of multinucleated cells in the liver. The literature has recorded only approximately one hundred cases in adults during the past two decades in whom malignancy is rarely associated with giant cell hepatitis. Our patient is a 66-year-old woman who was diagnosed with chronic lymphocytic leukemia (CLL) and subsequently developed syncytial giant cell hepatitis. This disease is usually linked to virus infection, autoimmune diseases, and drug medications. The association between CLL and giant cell hepatitis is rare, with only three cases reported. In most cases viral particles on electron microscopy are reported and the patients usually have a history of chemotherapy and hypogammaglobulinemia. Unlike such cases, our patient developed giant cell hepatitis in the absence of such confounding variables. The treatment for our patient was a high-dose corticosteroid and rituxan, with improvement in liver enzymes.
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Affiliation(s)
- Neil Gupta
- Yale University School of Medicine, New Haven, Connecticut,, USA
| | - Basile Njei
- Yale University School of Medicine, New Haven, Connecticut,, USA
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