Liver Pathology IV Flashcards by tcarlso 3

What is the underlying cause of hereditary hemochromatosis?

HFE gene mutation, causing changes in hepatocidin

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What are the ssx of hemachromatosis?

psychosis

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What is the pathophysiology of hereditary hemochromatosis?

Excess deposition of Fe in the liver d/t increased uptake

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What is the transport enzymes in the enterocytes that is regulated by heptacidin?

Ferroportin

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What is the M:F ratio of hereditary hemochromatosis?

7:1

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What are the two mutant alleles with hemochromatosis?

C282Y and H63D

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What are the early changes in hemochromatosis?

Fe deposits in the periportal hepatocytes and other cells

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What are the late changes in hemochromatosis?

Fe deposition in Kupffer cells, bile duct epi

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When do the severe ssx usually occur with hemochromatosis?

40s ish

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What level of serum Fe suggests hemochromatosis?

> 300 mg/dL

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What level of transferrin saturation suggests hemochromatosis?

> 2x normal

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What level of transferrin serum ferritin suggests hemochromatosis?

> 2x

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What are the two lab tests the demand thinking about hemochromatosis?

Elevated transferrin and ferritin

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What are the comorbidities of hemochromatosis?

DM
Caridomyopathy
Hypogonadism

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What are the skin changes with hemochromatosis?

gray pigmentation

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What is the cause of pseudogout in hemochromatosis?

build up of Fe in joints

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What are the pituitary changes with hemochromatosis?

Hypopituitarism

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What is the stain for Fe?

Prussian blue

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What CA are pts with hemochromatosis susceptible to?

Hepatocellular CA

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What are the histological changes in the liver with hemochromatosis?

Hepatocellular swelling

Fe stain in Kupffer cells

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What cause the DM in hemochromatosis?

Fe build up in the pancreas

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What are the causes of secondary hemochromatosis?

Transfusions
Hemochromatosis
Alimentary
Liver disease
Anemia
Sideroblastic anemia

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What is the cause of sideroblastic anemia?

Inability to incorporate Fe into Hb–causes ring cells

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What cells store the Fe in secondary hemochromatosis in the liver?

Kupffer cells

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What is the cause of Wilson's disease?

AR disorder d/t mutation in ATP7B Cu-transporter, causing deposition of Cu in the liver

What is Cu needed for?

- Pigmentation - Neurotransmitter production - CT synthesis

What is the protein that hold Cu in the circulation?

Ceruloplasmin

How is Cu excreted?

Biliary system

What are the two organs that accumulate Cu in Wilson's disease?

Brain and liver

What are the ssx of Wilson's disease?

Psychosis | Parkinson's

What is the function of ATP7B?

Takes Cu in hepatocyte into the biliary system

What are the histological changes in Wilson's disease?

Steatosis Mallory bodies Nuclear vacuoles

What cause the parkinson's symptoms in hemochromatosis?

Destruction of Dopaminergic neurons

What are the kidney issues with Wilson's disease?

Aminoaciduria

What are the eye findings with Wilson's disease?

Kayser-Fleischer rings

What are the cardiac problems with Wilson's disease?

Cardiomyopathy

What are the childhood problems with Wilson's disease?

Hepatitis | Acute/fulminant hepatitis

What are the diagnostic labs for Wilson's disease? (3)

- Decreased serum ceruloplasmin - Increased hepatic copper - Increase urinary Cu

What is the stain for Cu?

Rhodanine stain

What is alpha-1-antitrypsin deficiency?

AR disorder in anti- Trypsin (protease), causing unregulated trypsin destruction

What is the treatment for Wilson's disease?

Chelating agents

What is the treatment for hemochromatosis?

Phlebotomy

What is the primary liver disease in children?

Alpha-1-antitrypsin deficiency

What is the protein that is misfolded in alpha-1-antitrypsin deficiency?

PiZ

What protein accumulates in alpha-1-antitrypsin deficiency? Where?

PiZ in hepatocytes

What are the ssx of alpha-1-antitrypsin deficiency?

Neonatal = hepatitis/cholestasis Adults = chronic hepatitis and cirrhosis

What is the treatment for alpha-1-antitrypsin deficiency?

Liver transplant

What are the diagnostic labs for alpha-1-antitrypsin deficiency?

- Low serum alpha 1AT | - Abnormal electrophoretic alphaAT

What is the stain that is useful for alpha-1-antitrypsin deficiency?

PAS

What is the cause of COPD with alpha-1-antitrypsin deficiency?

degradation of the alveolar sacs

What zone of the liver is affected first in alpha-1-antitrypsin deficiency?

Zone 1

What is nodular regenerative hyperplasia?

benign nodular regeneration all over the liver

What is focal nodular hyperplasia?

Frequent benign liver tumor, often discovered incidentally

What is nodular hyperplasia usually confused with?

Hepatocellular CA

In whom does focal nodular hyperplasia usually occur?

Women on birth control

What is the treatment for focal nodular hyperplasia?

No issues with it.

What are the gross findings with focal nodular hyperplasia?

Solitary mass | Central scar

Are focal nodular hyperplasia usually encapsulated?

No, but well demaracated

What is the difference between nodular regenerative hyperplasia, and hepatocellular CA?

Nodules WITHOUT fibrous septa

What does nodular regenerative hyperplasia cause? What is the treatment for this?

Non-cirrhotic portal HTN | Shunt/Endoscopic treatment/beta blockers

What is the most common complication of nodular regenerative hyperplasia? How do they fix this surgically?

Portal HTN | TIPS shunt

What is the most common benign hepatic tumor?

Hemangioma

What drug is correlated with hepatic adenomas?

Oral contraceptives or steroids

What are cavernous hemangiomas?

composed of dilated, variably-sized vascular spaces lined by flattened endothelial cells and supported by connective tissue septa. Thrombi are often present.

Who usually have liver cell adenoma?

Young women on oral contraceptives, or men taking steroids

What glycogen storage diseases cause liver cell adenoma?

1 (Pompe's) and 3

Is there a capsule around the hepatic adenomas? Is there vessels within it?

Encapsulated without vessels

What is the most common primary malignant liver tumor?

Hepatocellular CA

What can cirrhosis lead to?

Hepatocellular CA

What are the aflatoxins that cause cause hepatocellular CA?

Fungal toxins

What is the pathophysiology with hepatocellular CA?

Proliferating hepatocytes d/t chronic insult

HBV is what type of virus? Genetic material? What are the things it has that causes hepatocellular CA?

Hepadnavirus dsDNA HBx inhibits p53

What is the pre-S mutation?

HBV surface antigen mutation that inhibit p26, Rb, other cell cycles. Found on some, but not all HBVs

What are the ssx of hepatocellular CA?

Painful hepatomegaly | Massive bleeding

What is the protein that is associated (high) with hepatocellular CA?

Alpha Fetoprotein >1000

Do fibrolamellar hepatocellular CA have a better or worse prognosis that hepatocellular CA? What are these?***

Better Broad thick bands of fibrous tissue that surround the malignant cells

What is cholangioCA?

Malignant primary tumor of the bile duct epithelium

What are the Risk factors for cholangioCA?

- Liver flukes (opisthorchis sinensis) | - Primary sclerosing cholangitis

What is a hepatoblastoma? What is the pathway that is thought to be mutated in this disease?

Uncommon malignant liver cancer occurring in infants and children and composed of tissue resembling fetal liver cells, mature liver cells, or bile duct cells. They usually present with an abdominal mass. The disease is most commonly diagnosed during a child's first three years of life. Caused by a WNT pathway mutation

What is the most common primary malignant tumor of children?

Hepatoblastoma

What is the lab that is elevated with hepatoblastomas? What if this lab is not elevated?

AFP--if not bad prognosis

What are angiosarcomas? In whom does the liver type occur in?

Malignant endothelial cells of blood vessels. If in the liver, usually caused by PVC workers

What are the predisposing factors to angiosarcomas?

Exposure to thorotrast | Vinyl chloride

What is the prognosis for angiosarcomas?

Very poor

What is the most common malignant tumor of the liver (NOT primary tumor)? What are the ssx of this?

Mets from the GI tract, or breast. ssx are usual liver problems

What are the histological characteristics of cholangiocarcinoma?

Tons of duct and glands

Liver Pathology IV Flashcards

(86 cards)