Transcript 50 year old man with history of Hodgkin’s lymphoma as a

CPC November 3, 2009
Charles Steenbergen
[email protected]
47 year old woman with
history of alcoholic cirrhosis,
diabetes, chronic pancreatitis,
and hepatocellular carcinoma,
who had liver transplant in
2/2008.
Developed
heart
failure, renal failure, and
dyspnea, and died 6/2008.
At autopsy, we found
• Massive ascites (4.9 liters, serosanguinous)
• Left pleural effusion (485 ml, serosanguinous)
• Transplanted liver with diffuse congestion
• Heart, 400 grams, with patchy LV fibrosis,
mild aortic atherosclerosis, and moderate
coronary atherosclerosis (< 50% stenosis)
Patchy interstitial fibrosis of the
left ventricular myocardium
Brown granular deposits in the
middle of cardiac myocytes
Massive iron deposition in cardiac myocytes
(Prussian blue stain for iron)
No evidence of extrahepatic biliary
obstruction or periportal damage
Centrilobular congestion and necrosis
Centrilobular fibrosis and
canalicular cholestasis
Iron deposition in reticuloendothelial cells
(Prussian blue stain for iron) and
canalicular bile plugs
Hyaline membranes in left lung, indicative
of diffuse alveolar damage (ARDS)
Fibrosis and calcification of the
pancreas
Microcalcifications within tubules
of kidneys
Myocardial iron deposition can be
associated with myocyte damage
and replacement fibrosis, which is
thought to be mediated by oxygen
free radical production catalyzed by
iron. This can explain the rapid
development of heart failure that
occurred in this patient.
At transplant, the explanted liver was
found to have marked iron deposition.
Hepatic iron deposition can be genetic
(hereditary hemochromatosis), but iron
accumulation can also occur in patients
with cirrhosis in the absence of HFE
mutations.
Iron deposition in liver
explants is a risk factor for myocardial
iron accumulation and heart failure.
(Transplantation 2009;87:1256).
A similar conclusion was reached in
an earlier study (Liver International
2005;25:513). Retrospective analysis
of three autopsy cases of end-stage
cirrhosis with marked hepatic iron
overload also showed iron deposition
in the heart and pancreas, as in the
current case. In the 2005 study, two
patients were heterozygous for HFE
mutations and one was wild-type.