Liver Path: Cholestatic/Neoplastic Disease Flashcards Preview

GI > Liver Path: Cholestatic/Neoplastic Disease > Flashcards

Flashcards in Liver Path: Cholestatic/Neoplastic Disease Deck (39):
1

What are causes of steatosis? (4)

Alcoholic liver disease
NAFLD
Ischemia
Inherited disorders: FAO defects, mitochondriopathies, galctosemia, fructose intolerance, CF, cholesterol storage disorder

2

Describe the histology of alcoholic liver disease (2)

Steatosis with pericentral sinusoidal (arachnoidal/chicken wire) fibrosis

Mallory denk bodies (balloon appearance with eosinophilic hyaline bodies)

3

NAFLD: describe the prevalence and histology

Most common cause of chronic liver disease in US (3-5% population)

Histological features in adults overlap with alcoholic liver disease

4

Describe the difference between hemochromatosis and hemosiderosis

Hemochromatosis: due to gene mutation in HFE, transferrin receptor, hepcidin or HJV-- it is hereditary

Hemosiderosis is secondary hemochromatosis due to other causes

5

Describe the causes of hemosiderosis (5)

Parenteral iron overload, Thalassemia
Increased oral intake
Chronic liver disease
Neonatal hemochromatosis

6

Describe histology for hereditary hemochromatosis

Using prussian blue stain, can see iron deposition in hepatocytes (it is golden-brown)

Also observe iron deposition in pancreas, heart, joints, endocrine organs

7

Describe histology of hemosiderosis

Iron accumulation mainly occurs in kuppfer cells

8

What is cause of neonatal hemochromatosis?

Prenatal onset: in-utero allimmune reaction of mother with formation of anti-liver antibodies resulting in liver/renal failure

9

Describe the intrahepatic causes of cholestasis (5)

Diffuse hepatocellular disease (sepsis, hepatitis, cirrhosis)
Canalicular membrane changes (drugs/pregnancy)
Genetic defects in cholestatic transporters (Dubin-Johnson, Rotor)
Primary biliary cirrhosis
Ductopenia

10

Describe extra hepatic causes of cholestasis (4)

Bile duct stones
Intra/extra biliary tumors
Sclerosing cholangitis
Biliary atresia

11

Compare and contrast primary biliary cirrhosis vs primary sclerosing cholangitis:

Age, gender, associated conditions, serology, radiology, duct lesion

Do it do it

12

Describe PBC: Age, gender, associated conditions, serology, radiology, duct lesion

Age: median=50yo
Gender: 90% female
Associated conditions: Sjogren, thyroid disease
Serology: AMA, ANA, ANCA
Radiology: normal
Duct lesion: Inflammatory destruction

13

Describe PSC: Age, gender, associated conditions, serology, radiology, duct lesion

Age: 30 yo
Gender: 70% male
Associated conditions: IBD, AIH
Serology: ANCA
Radiology: strictures and beading of large extra hepatic ducts
Duct lesion: onion skin fibrosis

14

Describe pathophysiology of primary biliary cirrhosis

Inflammatory destruction of interlobular bile ducts with granulomas leading to cirrhosis with ductopenia

15

Describe histology of PSC

Fibrous obliterative ductal lesions

Onion skinning fibrosis around destroyed bile duct

16

What are causes of ductopenia in adults? (3)

Immune: PBC, PSC, sarcoidosis, rejection, GVHD
Secondary: mechanical, drugs
Idiopathic

17

What are causes of ductopenia in pediatric populations? (4)

Syndromatic: Alagille syndrome
Idiopathic
Metabolic: A1AT deficiency
Secondary: biliary atresia

18

What is alagille syndrome?

Autosomal dominant disorder leading to pathology in liver, heart, skeleton, eye, face, kidney, vasculature

19

What are causes of obstructive cholestasis? (4)

Cholelithiasis (gallstones)
Malignancy of biliary tree of pancreas head
Strictures from surgery
Biliary atresia (children)

20

What are histological findings of extra hepatic bile duct obstruction? (4)

Portal fibrous expansion
Bile duct proliferation
Bile plugs
Feathery degeneration of hepatocytes

21

What histological finding occurs in ascending cholangitis due to gallstones?

Increased neutrophils

22

What are pathology findings in late duct obstruction? Histological and gross

Biloma: a bile filled cyst in liver
Biliary cirrhosis: due to extravasation of bile within liver

23

Hepatic Infarction: histology

Coagulative necrosis of hepatocytes with hyperemic rim on edge of necrosis

Can also observe infarct of major ducts of biliary tree

24

Describe causes of portal venous obstruction: both extrahepatic (4) and intrahepatic (2)

Extrahepatic: neonatal umbilical vein catheterization, intraabdominal sepsis, hypercoagulable disorders, pancreatitis

Intrahepatic: schistosomiasis, obliterative portal venopathy

25

What is the cause of Budd Chiari Syndrome?

What are the symptoms? (3)

Outflow obstruction due to hepatic vein thrombosis

Symptoms of Budd Chiari include hepatomegaly, ascites and liver dysfunction

26

What is sinusoidal obstruction syndrome (aka veno-occlusive disease)?

Endothelial injury to sinusoids and terminal hepatic venules

Observed in patients post stem cell tx and receiving chemo

27

What are histological findings in sinusoidal obstruction syndrome? (3)

Centrilobular congestion
Hepatocellular necrosis
Obliteration of small hepatic veins

28

How does liver appear in CHF? (3)

Congestion features similar to venous obstruction confined to zone 3
Nutmeg liver
Later as sinusoidal fibrosis occurs, septa connect hepatic and portal veins, forming cardiac sclerosis/cirrhosis

29

What is the cause of echninococcal/hyatid cysts?

What is concern during removal?

Larval stages of tapeworm

Surgical removal risks rupture which can result in fever, anaphylaxis

30

What are other types of cysts observed in liver? (2)

Simple cysts-- bile duct cyst
Polycystic liver disease

31

What are the two types of vascular tumors in liver?

How do they present?

Carvernous hemangioma==>most common liver tumor in adults; often asymptomatic or produces abd pain

Infantile hemangioendothelioma==>most common liver tumor in children

32

What is hepatocellular adenoma associated with? (2)

OCP
Metabolic diseases (i.e glycogen storage disease)

33

Describe focal nodular hyperplasia--
Epidemiology (prevalence, demographic)
Gross appearance

Most frequent solid lesion

3% of population, young females
Surrounding liver is normal; there is a central stellate scar

34

What are the types of malignant epithelial liver tumors? (3)

HCC
Cholagniocarcinoma
Hepatoblastoma

35

Describe the epidemiology of HCC: prevalence, geography, RFs

HCC=5% all cancers
Highest incidence in asian countries with high HBV prevalence

RFs: HBV, HCV, Aflatoxin, alcohol, metabolic diseases...anything that causes cirrhosis

36

What are the prognostic factors for HCC? (4)

What is the prognosis for HCC?

Vascular invasion
Differentiation
Number/size of nodules
Extra hepatic spread (IVC, lungs, regional lymph nodes)

Prognosis is terrible (

37

What is precursor for HCC?

Dysplastic lesions

38

What is fibrolamellar carcinoma?

A variant of HCC (makes up 5%)
Typically appears

39

Hepatoblastoma: how does it present? what is prognosis?

Presents with asymptomatic abdominal mass in children

Better prognosis than HCC