Syncytial giant-cell hepatitis: Sporadic Hepatitis with Distinctive Pathological Features, a Severe Clinical Course, and Paramyxoviral Features

M. James Phillips*, Lawrence M. Blendis, Siria Poucell, Jacqueline Patterson, Martin Petric, Eve Roberts, Gary A. Levy, Riccardo A. Superina, Paul D. Greig, Ross Cameron, Bernard Langer, Robert H. Purcell

*Corresponding author for this work

Research output: Contribution to journalArticle

174 Citations (Scopus)

Abstract

Background and Methods. We describe a new form of hepatitis, occurring in 10 patients over a period of six years, characterized clinically by manifestations of severe hepatitis, histologically by large syncytial giant hepatocytes, and ultrastructurally by intracytoplasmic structures consistent with paramyxoviral nucleocapsids. Results. The patients ranged in age from 5 months to 41 years. The tentative clinical diagnosis before biopsy was non-A, non-B hepatitis in five patients and autoimmune chronic active hepatitis in the others. Five patients underwent liver transplantation; the others died. The diagnosis of syncytial giant-cell hepatitis was established pathologically. The liver cords were replaced in all 10 patients by syncytial giant cells with up to 30 nuclei. In 8 of the 10 the cytoplasm contained pleomorphic particles of 150 to 250 μm, filamentous strands, and particles of 14 to 17 nm with peripherally disposed spikes resembling paramyxoviral nucleocapsids. Structures resembling degenerated forms were found in the other two patients. One of two chimpanzees injected with a liver homogenate from the index patient had an increase in the titer of paramyxoviral antibodies, probably an anamnestic reaction to previous paramyxoviral infection, suggesting that a paramyxoviral antigen but not viable virus was present in the liver homogenate. Conclusions. Although further virologic studies will be required for precise classification, we believe that paramyxoviruses should be considered in patients with severe sporadic hepatitis. (N Engl J Med 1991; 324:455–60.).

Original languageEnglish (US)
Pages (from-to)455-460
Number of pages6
JournalNew England Journal of Medicine
Volume324
Issue number7
DOIs
StatePublished - Feb 14 1991

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Giant Cells
Hepatitis
Nucleocapsid
Liver
Autoimmune Hepatitis
Pan troglodytes
Chronic Hepatitis
Liver Transplantation
Hepatocytes
Cytoplasm
Viruses
Biopsy
Antigens
Antibodies
Infection

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Phillips, M. James ; Blendis, Lawrence M. ; Poucell, Siria ; Patterson, Jacqueline ; Petric, Martin ; Roberts, Eve ; Levy, Gary A. ; Superina, Riccardo A. ; Greig, Paul D. ; Cameron, Ross ; Langer, Bernard ; Purcell, Robert H. / Syncytial giant-cell hepatitis : Sporadic Hepatitis with Distinctive Pathological Features, a Severe Clinical Course, and Paramyxoviral Features. In: New England Journal of Medicine. 1991 ; Vol. 324, No. 7. pp. 455-460.
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abstract = "Background and Methods. We describe a new form of hepatitis, occurring in 10 patients over a period of six years, characterized clinically by manifestations of severe hepatitis, histologically by large syncytial giant hepatocytes, and ultrastructurally by intracytoplasmic structures consistent with paramyxoviral nucleocapsids. Results. The patients ranged in age from 5 months to 41 years. The tentative clinical diagnosis before biopsy was non-A, non-B hepatitis in five patients and autoimmune chronic active hepatitis in the others. Five patients underwent liver transplantation; the others died. The diagnosis of syncytial giant-cell hepatitis was established pathologically. The liver cords were replaced in all 10 patients by syncytial giant cells with up to 30 nuclei. In 8 of the 10 the cytoplasm contained pleomorphic particles of 150 to 250 μm, filamentous strands, and particles of 14 to 17 nm with peripherally disposed spikes resembling paramyxoviral nucleocapsids. Structures resembling degenerated forms were found in the other two patients. One of two chimpanzees injected with a liver homogenate from the index patient had an increase in the titer of paramyxoviral antibodies, probably an anamnestic reaction to previous paramyxoviral infection, suggesting that a paramyxoviral antigen but not viable virus was present in the liver homogenate. Conclusions. Although further virologic studies will be required for precise classification, we believe that paramyxoviruses should be considered in patients with severe sporadic hepatitis. (N Engl J Med 1991; 324:455–60.).",
author = "Phillips, {M. James} and Blendis, {Lawrence M.} and Siria Poucell and Jacqueline Patterson and Martin Petric and Eve Roberts and Levy, {Gary A.} and Superina, {Riccardo A.} and Greig, {Paul D.} and Ross Cameron and Bernard Langer and Purcell, {Robert H.}",
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Phillips, MJ, Blendis, LM, Poucell, S, Patterson, J, Petric, M, Roberts, E, Levy, GA, Superina, RA, Greig, PD, Cameron, R, Langer, B & Purcell, RH 1991, 'Syncytial giant-cell hepatitis: Sporadic Hepatitis with Distinctive Pathological Features, a Severe Clinical Course, and Paramyxoviral Features', New England Journal of Medicine, vol. 324, no. 7, pp. 455-460. https://doi.org/10.1056/NEJM199102143240705

Syncytial giant-cell hepatitis : Sporadic Hepatitis with Distinctive Pathological Features, a Severe Clinical Course, and Paramyxoviral Features. / Phillips, M. James; Blendis, Lawrence M.; Poucell, Siria; Patterson, Jacqueline; Petric, Martin; Roberts, Eve; Levy, Gary A.; Superina, Riccardo A.; Greig, Paul D.; Cameron, Ross; Langer, Bernard; Purcell, Robert H.

In: New England Journal of Medicine, Vol. 324, No. 7, 14.02.1991, p. 455-460.

Research output: Contribution to journalArticle

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T1 - Syncytial giant-cell hepatitis

T2 - Sporadic Hepatitis with Distinctive Pathological Features, a Severe Clinical Course, and Paramyxoviral Features

AU - Phillips, M. James

AU - Blendis, Lawrence M.

AU - Poucell, Siria

AU - Patterson, Jacqueline

AU - Petric, Martin

AU - Roberts, Eve

AU - Levy, Gary A.

AU - Superina, Riccardo A.

AU - Greig, Paul D.

AU - Cameron, Ross

AU - Langer, Bernard

AU - Purcell, Robert H.

PY - 1991/2/14

Y1 - 1991/2/14

N2 - Background and Methods. We describe a new form of hepatitis, occurring in 10 patients over a period of six years, characterized clinically by manifestations of severe hepatitis, histologically by large syncytial giant hepatocytes, and ultrastructurally by intracytoplasmic structures consistent with paramyxoviral nucleocapsids. Results. The patients ranged in age from 5 months to 41 years. The tentative clinical diagnosis before biopsy was non-A, non-B hepatitis in five patients and autoimmune chronic active hepatitis in the others. Five patients underwent liver transplantation; the others died. The diagnosis of syncytial giant-cell hepatitis was established pathologically. The liver cords were replaced in all 10 patients by syncytial giant cells with up to 30 nuclei. In 8 of the 10 the cytoplasm contained pleomorphic particles of 150 to 250 μm, filamentous strands, and particles of 14 to 17 nm with peripherally disposed spikes resembling paramyxoviral nucleocapsids. Structures resembling degenerated forms were found in the other two patients. One of two chimpanzees injected with a liver homogenate from the index patient had an increase in the titer of paramyxoviral antibodies, probably an anamnestic reaction to previous paramyxoviral infection, suggesting that a paramyxoviral antigen but not viable virus was present in the liver homogenate. Conclusions. Although further virologic studies will be required for precise classification, we believe that paramyxoviruses should be considered in patients with severe sporadic hepatitis. (N Engl J Med 1991; 324:455–60.).

AB - Background and Methods. We describe a new form of hepatitis, occurring in 10 patients over a period of six years, characterized clinically by manifestations of severe hepatitis, histologically by large syncytial giant hepatocytes, and ultrastructurally by intracytoplasmic structures consistent with paramyxoviral nucleocapsids. Results. The patients ranged in age from 5 months to 41 years. The tentative clinical diagnosis before biopsy was non-A, non-B hepatitis in five patients and autoimmune chronic active hepatitis in the others. Five patients underwent liver transplantation; the others died. The diagnosis of syncytial giant-cell hepatitis was established pathologically. The liver cords were replaced in all 10 patients by syncytial giant cells with up to 30 nuclei. In 8 of the 10 the cytoplasm contained pleomorphic particles of 150 to 250 μm, filamentous strands, and particles of 14 to 17 nm with peripherally disposed spikes resembling paramyxoviral nucleocapsids. Structures resembling degenerated forms were found in the other two patients. One of two chimpanzees injected with a liver homogenate from the index patient had an increase in the titer of paramyxoviral antibodies, probably an anamnestic reaction to previous paramyxoviral infection, suggesting that a paramyxoviral antigen but not viable virus was present in the liver homogenate. Conclusions. Although further virologic studies will be required for precise classification, we believe that paramyxoviruses should be considered in patients with severe sporadic hepatitis. (N Engl J Med 1991; 324:455–60.).

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