Thursday, February 16, 2006 - 7:30 PM
Mary Kay Washington
Vanderbilt University Medical Center
Disclosure: The speakers have indicated they have nothing to disclose.
Clinical histories are printed below.
Click on the case numbers for text and references of each case.
Click on each slide thumbnail image for an enlarged view
University of California
San Diego, CA
The patient is currently a 9 year old obese Hispanic boy who had initially been referred to hepatology
clinic at the age of 4 when hepatomegaly was discovered by his pediatrician during a well care visit.
A liver biopsy was performed and showed steatosis . Etiologies of liver disease other than steatosis
were excluded. No specific treatment was initiated except for dietary counseling and discussion of
The patient continued to receive care from
his pediatrician but recently returned to hepatology for followup of his liver condition, because of
excess weight and a concern for the development of diabetes.
The patient was born at 8 months gestation after an uneventful prenatal course. He
weighed 5 lbs 4 oz at birth. Developmental milestones were met. When he was initially referred to
hepatology at the age of 4, he was noted to be "overweight". The patient has mild asthma. His
dietary intake is notable for 2-3 regular sodas per day, candy, fried cheese snacks and ice cream on a
daily basis, fast food meals twice a week large portion sizes and 4-5 hours of television viewing per
day. He does not regularly exercise.
The patient's father is morbidly obese and has hypertension and type 2 diabetes. The patient's mother
is in good health. Maternal and paternal relatives have diabetes, hypertension, high cholesterol.
Inhaled albuterol PRN
At the time of his return to Heptology Clinic at the age of 9, the patient weighed 55.7 kg and
measured 135 cm in height. BMI was 30.56. The remainder of the physical exam was normal except for
the presence of severe acanthosis nigricans around the neck and in the axilla bilaterally.
Significant laboratory abnormalities include ALT 383, AST 257, GGT 171. Fasting glucose was 92.
A second liver biopsy was performed. On the
basis of the results, the patient was eligible for and consented to be part of an NIH sponsored
treatment trial and is currently enrolled in the trial.
Both liver biopsies are included for review.
University of California
Los Angeles, CA
The patient is a 22 year old woman, G3P1, s/p C-section for full-term pregnancy, transferred to UCLA
Medical center with deep jaundice and acute liver failure. There was no prior history of liver
disease. She was approximately 38 weeks gravid when she noted ankle swelling, and when elevated blood
pressure was detected. There was no pruritus and no alteration in mental status. She presented to a
local Medical Center (1 week prior to transfer) with an approximately a 1-week history of increasing
jaundice. Upon admission, she was noted to be deeply jaundice and anemic. The fetus was bradycardic.
A STAT C-section was performed, and the baby has reportedly been doing well post-delivery.
Following delivery, she developed hypotension
and was transferred to the ICU for close monitoring. There, she was given PRBC's and FFP for anemia
and coagulopathy. She remained afebrile. On post-operative day #1, she was noted to be somewhat
lethargic. Her serum bilirubin continued to rise, though her transaminases remained low. She
developed progressive thrombocytopenia to 43,000. She was started on IV antibiotics. Over the
subsequent days, her coagulopathy progressed with increasing jaundice requiring supportive
transfusions. Her renal function progressively worsened. She also developed increasing abdominal
distention and discomfort. Paracentesis revealed bloody fluid without obvious infection. In view of
her worsening clinical picture, she was transferred for a higher level of care. The clinical
differential diagnosis included acute fatty liver of pregnancy vs. HELLP. After transfer,
coagulopathy worsened, renal function deteriorated, encephalopathy developed, and the bilirubin
continued to rise. The AST and ALT remained mildly elevated. The patient underwent orthotopic liver
transplantation approximately 2 after initial presentation and one week after C-section.
| ||OLT-14 days ||OLT-7days ||OLT - 1day|
|AST ||51 ||81 ||87|
|ALT ||38 ||32 ||41|
|Total bili ||20.7 ||34.3 ||41.5|
|Alk phos ||333 ||252 ||173|
|HCT ||30.5 || || |
|PLTS ||75 || || |
|WBC ||11.7 ||30.6 || |
Raouf E. Nakhleh
A 60yo Caucasian male with end-stage liver disease secondary to alcohol use undergoes liver
transplantation. At the time of transplant, the patient had refractory ascites requiring a TIPS
procedure a year prior. The native liver weight was 1210gm and was diffusely nodular. Near the hilum
a prominent 1.2cm tan nodule was present and is seen on the slides.
Case 3 - Figure 10 - High power view of spindle cells
Case 3 - Figure 11 - Immunohistochemical stain for HMB-45 shows diffuse positivity within the nodule.
Case 3 - Figure 12 - HMB 45. A granular cytoplasmic staining pattern is present in a majority of the lesional cells.
Case 3 - Figure 13 - Immunohistochemical stain for actin shows patchy staining within the nodule.
Case 3 - Figure 14 - Actin. Epithelioid and spindle cells within the nodule are immunoreactive for actin.
National Institutes of Health
The patient is a 23 year old man with a history of recurrent pulmonary, hepatic and bone infections
since young childhood. These infections have required repeated hospitalizations and chronic
administration of antibiotics and antifungals. This time he was admitted for investigation of
sinusitis and during the work-up was noted to have an elevated liver associated enzymes (Alkaline
phosphatase 866 (range 37-116), ALT 52 (range 6-41), AST 37 (range 9-34) and total bilirubin of 0.6
mg/dL). Imaging using ultrasound, CT and MRI showed multiple hepatic lesions consistent with
abscesses. A CT guided aspirate grew Staph. aureus and Strep. mitis. He was started on Ceftriaxone,
Vancomycin and Rifampin and discharged, but returned one week later with right upper quadrant pain,
fatigue and chills. He was taken to surgery where drains were placed and wedge resections of liver
were performed. One of the specimens was a wedge of liver measuring 7 x 4.5 x 3 cm. Sectioning
revealed multiple small firm white nodules measuring up to 0.4 cm, but no frank abscesses. Hemorrhage
and fibrosis was noted on one edge of the specimen. The section submitted for review was selected
from an area without obvious gross parenchymal lesions.
Case 4 - Figure 4 - One of numerous collections of pigmented macrophages (40x, H&E)
Case 4 - Figure 5 - Scattered granulomas were seen. This one may have been located in or near a portal area (20x, H&E)
Case 4 - Figure 6 - Another example of the granulomatous inflammation (20x, H&E)
University of Chicago
This 57-year-old male was diagnosed with chronic HBV hepatitis in 1991. Laboratory evaluation at that
time revealed a positive serum HBsAg test but a negative HBeAg test. A liver biopsy in 1994
documented the presence of cirrhosis. An abdominal ultrasound examination and CT scan performed in
1998 revealed two nodules that were more than 4 cm in greatest dimension, as well as several smaller
lesions. None of the lesions was regarded as radiographically diagnostic of hepatocellular carcinoma.
The serum AFP at that time was 30 ng/ml. A percutaneous needle biopsy of the largest nodule was read
as atypical macroregenerative nodule (borderline lesion). The patient was treated with Lamivudine and
followed with serial MRI examinations and serum AFP levels. The two large lesions remained
essentially unchanged and the serum AFP level remained stable.
On 4/3/01 the patient underwent orthotopic liver transplantation. Examination of the explanted native
liver revealed cirrhosis with six distinct nodules, including 6.0 cm and 5.0 cm masses. Sections
revealed that two of the smaller lesions (3.5 and 3 cm) were well-differentiated hepatocellular
carcinomas. There were also two macroregenerative nodules. Sections of the two largest lesions were
more difficult to precisely classify. A section from the largest mass is submitted for your review.
The patient has had no evidence of recurrent hepatocellular carcinoma in the post-transplant period,
with last follow-up on 3/6/05.