Autoimmune hepatitis with giant-cell transformation

Department of Gastroenterology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico.
Annals of hepatology: official journal of the Mexican Association of Hepatology (Impact Factor: 2.07). 01/2009; 8(1):68-70.
Source: PubMed


Giant-cell hepatitis (GCH), also known as postinfantile or syncytial giant cell hepatitis, is a frequent pattern of liver injury in the neonate, primarily seen in the first three months of life. Few cases in adults have been reported, some of them associated to autoimmune diseases such as autoimmune hepatitis.
We present a case of autoimmune hepatitis with giant cell transformation in a 39 year old male with polyarthritis. We discuss his clinical presentation and course. We made a review of the literature of agents associated to this diagnosis, the mechanisms involved in the formation of giant hepatocytes, the histological findings, clinical course, treatment options and prognosis of this rare entity.
In conclusion, the clinical course varies from normalization of hepatic histology to progression to cirrhosis and liver failure. The prognosis is dictated by the underlying liver disease and in the setting of autoimmune hepatitis the clinical course is usually severe with most of the patients progressing to cirrhosis. Prolonged treatment with corticosteroids and immunosuppressants is usually effective in rendering the cirrhosis inactive.

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Available from: Braulio Martínez-Benítez, Jul 27, 2015
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    • "1 Good (responded to immunossuppresion) [11] SLE 2 Moderate [2] [12] Autoimmune hemolytic anemia 3 P o o r [13] [14] [15] PSC + AIH 2 Moderate (rapid onset of cirrhosis) Good (mild hepatitis) [16] [17] AIH + polyarthritis 1 Moderate (early cirrhosis) [18] AIH + polyarteritis 1 Moderate (early cirrhosis) [19] AIH + UC 1 Moderate (early cirrhosis) [20] PBC 2 1 Poor, (liver failure) 1 moderate (early cirrhosis) [21] [22] AIH II 1 Poor (died) [23] Viral HAV 4 Poor (fatal liver failure) [1, 24–26] HEV 1 Good (mild hepatitis) [27] HBV 3 Good (1 acute hepatitis, 2 chronic hepatitis) [2] [7] HCV 22 Good (chronic hepatitis) [5, 28–30] EBV 3 Poor (fatal liver failure) [31] [32] [33] Paramyxoviruses 13 Poor (7 fatal liver failure, 6 died) [3] [26] [34] [35] HIV + HCV 2 Good (chronic hepatitis) [30] HIV 2 Good (chronic hepatitis) [36] HHV-6A 1 Good (chronic hepatitis) [27] CMV 1 Poor (acute liver failure with underlying Wilson's diseases) [37] Hypereosinophilia 3 2 Poor (liver failure) 1 Good [24] [32] [38] CLL 3 2 Poor (liver failure) 1 Good (responded to immunosuppression) [39] Posttransplant 10 Poor (recurrent disease, mostly required retransplant) [40] [41] [42] [43] Associations with three eosinophilia cases were reported. Two of them had fatal disease course [24] [38], and one had better outcome [32]. "
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    ABSTRACT: Giant cell hepatitis is common manifestation in pediatric liver diseases, but quite uncommon in adults, only about 100 cases reported in the English literature in the last two decades. Data for the present review were identified by a structured PubMed/MEDLINE search from 1963 to December 2012, using keywords postinfantile giant cell hepatitis (PIGCH), adult giant cell hepatitis, and syncytial giant cell hepatitis in adults and liver. We report a case of postinfantile giant cell hepatitis along with the review related to the etiology and respective outcome, as the literature in the last 20 years suggests. This condition is probably due to idiosyncratic or cytopathic response of individual to various hepatocytic stimuli. It is purely a histomorphological diagnosis and does not establish the etiology. Autoimmune liver diseases are most common etiology, in around 40% of cases, but various viruses, drugs, posttransplant condition, and other causes also have been reported. Prognosis depends upon the etiology. In this paper, we emphasized various causative factors of PIGCH and their respective outcome in patients affected by them. We also highlighted the possible pathogenesis and histopathological spectrum of this entity on the basis of description given in various studies and our limited experience of few cases.
    Hepatitis research and treatment 03/2013; 2013:601290. DOI:10.1155/2013/601290
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    ABSTRACT: The aim of this study is to determine whether amitotic division or nuclear proliferation is involved in the formation of giant cells (GCs) in giant cell hepatitis (GCH). Liver sections from 18 pediatric patients with idiopathic infantile GCH and 12 patients with postinfantile GCH were evaluated for the expression of proliferating cell nuclear antigen (PCNA) and human histone 3 (H3) mRNA, transforming growth factor-alpha (TGF-α), TGF-β1, hepatocyte growth factor (HGF), and epidermal growth factor receptor (EGFR). Proliferation markers were detected in 1% to 80% in the nuclei of GC and non-GC hepatocytes in 10 of 18 (56%) infantile GCH biopsies and 11 of 12 (92%) postinfantile GCH biopsies, but not in normal liver. The expression of proliferation markers in GCs paralleled that in non-GC hepatocytes (P < 0.05 for both markers). TGF-α and EGFR were detected in both GCs (9/29 and 4/30 patients with infantile or postinfantile GCH, respectively) and non-GC hepatocytes (15/29 and 11/30 patients with infantile or postinfantile GCH, respectively). TGF-β1 and HGF were detected mainly in sinusoidal cells in 20 of 29 and 10 of 30 patients with infantile or postinfantile GCH, respectively; the expression of HGF was positively correlated with PCNA and H3 mRNA in non-GC hepatocytes and with H3 mRNA in GCs (P < 0.01). Hepatic expressions of nuclear proliferation markers and growth factors were similar in infantile and postinfantile GCH, nuclear proliferation markers were detected in both GCs and non-GC hepatocytes in a high proportion of patients, and expression of HGF correlated positively with the proliferation markers. These data indicate that nuclear proliferation may contribute to the pathogenesis of GCs in at least a proportion of patients with GCH. A model for the pathogenesis of GCH is proposed.
    Journal of pediatric gastroenterology and nutrition 12/2011; 52(1):65-72. DOI:10.1097/MPG.0b013e3181f85a87 · 2.63 Impact Factor
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