1. P R E S E N T E D B Y
M A H A E L S A B A AW Y
A S S O C I A T E P R O F E S S O R O F V
N L I
M E N O F I A U N I V E R S I T Y
2 0 1 5
2. Giant cell hepatitis or syncytial hepatitis
is a well known form of hepatitis only
to pediatricians
The adult form of this disease entity
which is nominated postinfantile giant
cell hepatitis (PIGCH) is an infrequent
presentation of liver disease in adults.
3. Only 100 cases of PIGCH were identified by a
structured PubMed/MEDLINE search from
1963 up to December 2012, with prevalence
rate of 0.1 to 0.25 %.
It is a purely histomorphological diagnosis.
4. PATHOGENESIS
This condition is probably due to idiosyncratic
or cytopathic response of individual to various
hepatocytic noxious stimuli.
Infection and autoimmunity are the
two evidence based milestones of PGCH
pathogenesis
5. TILL NOW; ALL REPORTS OF PIGCH HAD COUPLED
THIS DISEASE ENTITY TO:
CLL
CML
Lymphoma
AH
AHA
Polyarteritis
nodosa
PSC
PPC
Recurrence
after ltx
6. representing more than 40% of PIGCH
associations, AUTOIMMUNE DISORDERS
SHOULD BE THE first diagnostic
probability to be considered dealing
with a case of giant cell hepatitis.
8. VIRAL INFECTIONS PAIRED TO OCCURRENCE OF PIGCH
• A,B,D and E
• Strong association with C
solely or with HIV infection.
HEPETOTOPIC
• Epstein-Barr virus (EBV,
Cytomegalovirus, paramyxo-
like virus, and HIV
NON
HEPETOTOPIC
9. Clinical presentations and outcome
scenarios of PIGCH mainly relies upon the
offending etiological stimulus.
With about 50% survival rates this lethal
disease entity may present with acute :
fulminant to chronic hepatitis, up to
cirrhosis, and subacute to fulminant
hepatic failure .
10. Histopathological examination is the core of
PIGCH diagnosis with the characteristic giant
cell transformation of hepatocytes.
They are always the only apprehensive signal
of this disorder.
The multinuclear hepatitis is said to be due to
either fusion of hepatocytes or nuclear
proliferation without cell division.
12. PATHOLOGICAL FINDING
Lymphocytic infiltrate, massive necrosis, bridging
necrosis, “activated” perisinusoidal cells, bilirubin
stasis, and Mallory hyaline bodies, often
associated with neutrophilic infiltrate and severe
fibrosis are the most common histopathological
signs detected in liver biopsies of those patients
with PIGCH .
13. TREATMENT
Treatment of the noxious stimulus
Prolonged treatment with corticosteroids and
immunosuppresants is usually effective in
rendering cirrhosis inactive if it ever happens
In HCV CASES Pegylated interferon and
ribavirin combination treatment had led to
histological resolution with biochemical
improvement, even in the absence of HCV-RNA
clearance
14. CASE REPORTED IN NLI
An adult male presented with mixed hyperbilirubinemia
and acute hepatitis like picture (mixed hepatocellular and
cholestatic). He had no history of prior toxic exposure
with negative serology for both autoimmune and viral
markers.
Giant cell hepatitis was astonishingly proved by Liver
biopsy.
The search for the noxious stimulus for this disorder was
exhausting to the whole team without any relevance.
15. Endoscopic retrograde cholangiopancreatography
(ERCP) had shown evidence of extrahepatic
cholestasis with picture of obstructed thickened
common bile duct.
Plastic stenting was the procedure of choice for
obstruction relief.
Meanwhile the patient reported spontaneous
relief of the hepatitis status.
16. Brush cytology of the thickened distal end
of common bile duct (CBD) was practically
problematic, hence: Endoscopic ultrasound
(EUS) with biopsy was recommended;
surprisingly detected no abnormality with
slipped stent.
17. The new message to be sent from our
institute is that: PIGCH COULD
PRESENT ISOLATED WITHOUT ANY
ASSOCIATIONS.
We reported the case and publication
is pending
18. CONCLUSIONS
Two notions to be kept on mind on dealing
with a case of hepatitis of unknown origin;
PIGCH MIGHT be one of the probable
diagnostic potentials coming up to mind.
Liver biopsy should be considered as a
nessiccity whenever possible in those
patients.
