Abstract
AIM--To determine the composition of the inflammatory infiltrate and to check for the presence of cytomegalovirus (CMV) and Epstein-Barr virus (EBV) in nine cases of post-infantile giant cell hepatitis. METHODS--The clinical, serological, and histological features of the nine cases were reviewed. Immunohistochemistry was used on liver biopsy specimens from six cases to: (i) characterise the lymphocytic infiltrate; (ii) assess the monocyte/macrophage response; (iii) detect "activated" perisinusoidal cells; and (iv) detect CMV and EBV antigens. Electron microscopic examination was carried out in two cases. RESULTS--Four patients had serological features suggestive of autoimmune chronic active hepatitis; in the other five cases the aetiology was obscure. Two patients presented with neurological symptoms. Hepatitis resolved completely in one patient; two patients showed clinical improvement; and one remained stable. Cirrhosis developed in three patients, one of whom proceeded to liver transplantation, and three patients died. Portal inflammation was present in all cases and lymphocytic piecemeal necrosis in eight cases, but intra-acinar inflammation associated with hepatocyte necrosis was observed in only five cases. The inflammatory infiltrate was composed predominantly of T lymphocytes; an increase in monocyte/macrophage cells was also observed. Mallory bodies, often with associated neutrophilic infiltrate, were present in four cases, and bilirubinostasis was a feature in four cases. "Activated" perisinusoidal cells were present, especially in relation to areas of inflammation, necrosis, and fibrosis. There was severe fibrosis or cirrhosis in five cases. Paramyxoviral nucleocapsids were not seen in the two cases examined ultrastructurally. CONCLUSIONS--Post-infantile giant cell hepatitis should be viewed as a heterogeneous clinical and aetiological entity encompassing cases of hepatitis with extensive giant cell hepatocyte transformation.
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- ATERMAN K. Neonatal hepatitis and its relation to viral hepatitis of mother. A review of the problem. Am J Dis Child. 1963 Apr;105:395–416. doi: 10.1001/archpedi.1963.02080040397014. [DOI] [PubMed] [Google Scholar]
- Bach N., Thung S. N., Schaffner F. The histological features of chronic hepatitis C and autoimmune chronic hepatitis: a comparative analysis. Hepatology. 1992 Apr;15(4):572–577. doi: 10.1002/hep.1840150403. [DOI] [PubMed] [Google Scholar]
- Coe R. O., Bull F. E. Cirrhosis associated with methotrexate treatment of psoriasis. JAMA. 1968 Nov 11;206(7):1515–1520. [PubMed] [Google Scholar]
- Devaney K., Goodman Z. D., Ishak K. G. Postinfantile giant-cell transformation in hepatitis. Hepatology. 1992 Aug;16(2):327–333. doi: 10.1002/hep.1840160208. [DOI] [PubMed] [Google Scholar]
- Dubin H. V., Harrell E. R. Liver disease associated with methotrexate treatment of psoriatic patients. Arch Dermatol. 1970 Nov;102(5):498–503. [PubMed] [Google Scholar]
- ENDERS J. F., McCARTHY K., MITUS A., CHEATHAM W. J. Isolation of measles virus at autopsy in cases of giant-cell pneumonia without rash. N Engl J Med. 1959 Oct 29;261:875–881. doi: 10.1056/NEJM195910292611801. [DOI] [PubMed] [Google Scholar]
- Elsner B. Ultrastructural studies in giant cell transformation of the liver. Medicina (B Aires) 1973 May-Jun;33(3):248–254. [PubMed] [Google Scholar]
- Kumar A., Minuk G. Y. Postinfantile giant cell hepatitis in association with hypereosinophilia. Gastroenterology. 1991 Nov;101(5):1417–1419. doi: 10.1016/0016-5085(91)90096-4. [DOI] [PubMed] [Google Scholar]
- Lau J. Y., Koukoulis G., Mieli-Vergani G., Portmann B. C., Williams R. Syncytial giant-cell hepatitis--a specific disease entity? J Hepatol. 1992 May;15(1-2):216–219. doi: 10.1016/0168-8278(92)90039-r. [DOI] [PubMed] [Google Scholar]
- Mathew J., Hines J. E., Toole K., Johnson S. J., James O. F., Burt A. D. Quantitative analysis of macrophages and perisinusoidal cells in primary biliary cirrhosis. Histopathology. 1994 Jul;25(1):65–70. doi: 10.1111/j.1365-2559.1994.tb00599.x. [DOI] [PubMed] [Google Scholar]
- McILVANIE S. K., MACCARTHY J. D. Hepatitis in association with prolonged 6-mercaptopurine therapy. Blood. 1959 Jan;14(1):80–90. [PubMed] [Google Scholar]
- Montgomery C. K., Ruebner B. H. Neonatal hepatocellular giant cell transformation: a review. Perspect Pediatr Pathol. 1976;3:85–101. [PubMed] [Google Scholar]
- Pessayre D., Degos F., Feldmann G., Degott C., Bernuau J., Benhamou J. P. Chronic active hepatitis and giant multinucleated hepatocytes in adults treated with clometacin. Digestion. 1981;22(2):66–72. doi: 10.1159/000198597. [DOI] [PubMed] [Google Scholar]
- Phillips M. J., Blendis L. M., Poucell S., offterson J., Petric M., Roberts E., Levy G. A., Superina R. A., Greig P. D., Cameron R. Syncytial giant-cell hepatitis. Sporadic hepatitis with distinctive pathological features, a severe clinical course, and paramyxoviral features. N Engl J Med. 1991 Feb 14;324(7):455–460. doi: 10.1056/NEJM199102143240705. [DOI] [PubMed] [Google Scholar]
- Richey J., Rogers S., Van Thiel D. H., Lester R. Giant multinucleated hepatocytes in an adult with chronic active hepatitis. Gastroenterology. 1977 Sep;73(3):570–574. [PubMed] [Google Scholar]
- Thaler H. Post-infantile giant cell hepatitis. Liver. 1982 Dec;2(4):393–403. doi: 10.1111/j.1600-0676.1982.tb00839.x. [DOI] [PubMed] [Google Scholar]
- Thijs J. C., Bosma A., Henzen-Logmans S. C., Meuwissen S. G. Postinfantile giant cell hepatitis in a patient with multiple autoimmune features. Am J Gastroenterol. 1985 Apr;80(4):294–297. [PubMed] [Google Scholar]
- Witzleben C. L., Marshall G. S., Wenner W., Piccoli D. A., Barbour S. D. HIV as a cause of giant cell hepatitis. Hum Pathol. 1988 May;19(5):603–605. doi: 10.1016/s0046-8177(88)80213-2. [DOI] [PubMed] [Google Scholar]
- Witzleben C. L., Marshall G. S., Wenner W., Piccoli D. A., Barbour S. D. HIV as a cause of giant cell hepatitis. Hum Pathol. 1988 May;19(5):603–605. doi: 10.1016/s0046-8177(88)80213-2. [DOI] [PubMed] [Google Scholar]
- Yunis E., Agostini R. Syncytial giant-cell hepatitis. N Engl J Med. 1992 Jul 9;327(2):130–131. doi: 10.1056/NEJM199207093270218. [DOI] [PubMed] [Google Scholar]