Hemochromatosis and Selected Hepatic Neoplasms
Hemochromatosis = a disorder of iron metabolism with excess deposition of iron in the tissues, bronze skin pigmentation, hepatic cirrhosis, and diabetes mellitus.
IRON OVERLOAD SYNDROMES
- No physiological mech. for iron excretionÞ iron will accumulate in the body either from 1) increased intestinal absorption or from 2) parenteral administration
- Normal total body iron stores (3-4 g)
Hereditary Hemochromatosis
- characterized by: excessive iron absorption and toxic accumulation of iron in parenchymal cells
- Cirrhosis, diabetes, skin pigmentation, and cardiac failure
- Liver: enlarged and reddish brown – micronodular necrosis – Hepatocytes and bile duct cells filled with iron granules
- Body stores – increased to 20-40 g.
Secondary Iron Overload Syndromes
- Adults can absorb usually no more than 1mg per day of elemental iron, so diet irregularities usually err on the side of iron depletion.
- Usu accumulates in Macs first (kupffer cells) and then spills into parenchymal cells
- Body stores – increased much less tha hereditary Hemochromatosis
- Two forms
1) Increased iron absorption
Ø chronic liver disease, iron-loading anemias, porphyria cutanea tarda, dietary iron overload
Ø excessive medicinal iron.
2) Parenteral iron overload
Ø multiple iron tranfusions
Ø injectable medicinal iron
Differentiation (uses liver biopsy)
- Don’t believe that hemochromatosis = iron in hepatocytes and hemosiderosis = iron in macs (can happen any way)
- Quantative measure of total body iron is ONLY useful if there is little or no stainable iron, because this is never seen in untreated hereditary hemochromatosis
- It is necessary to get a 2cm needle biopsy for quantative iron determination in the liver.
NEOPLASMS OF THE LIVER
- most common benign neoplasm – hemangioma
- most common malignant neoplasm = metastatic cancer
Focal Nodular Hyperplasia
- generally solitary lesion in the lever substance or may be pedunculated
- central stellate scar
- unencapsulated, but well circumscribed
- central blood supply with smaller branches to the periphery
- hepatocytes 2 cell thick plates
- Clinical – most asymptomatic; rupture is rare; occur at any age
- Not related to oral contraceptive use
- Not a precursor to HCC.
Hepatocellular Adenoma
- Benign, usu solitary, 1/3 encapsulated
- Vasculature is more to the periphery of the lesion = higher chance of rupture with severe hemorrhage
- Portal tracts absent
- Looks a lot like well-differentiated HCC
- Clinical: almost all females in child bearing years, 2/3 palpable mass, ¼ present with circulatory collapse and hemoperitoneum from rupture
- Liver function tests – usu normal
- RELATED TO BIRTH CONTROL
- Probably not a precursor to Hepatocellular CA (HCC)
Hepatocellular Carcinoma (HCC)
- Two risk factors: cirrhosis and chronic HBV and HCV
- High Risk Countries: those with highest incidence of chronic HBV and HCV
Ø Africa and Asia
Ø HCV (chronic) makes the risk higher than HBV (chronic)
- Low Risk Countries (like U.S.): associated with alcoholic cirrhotic livers (90%)
- GROSS: large mass with satellite lesions; or scattered small lesions; May be green if producing bile
- HISTO: wide variation in differentiation
- Less than ½ of the cases have lymph node or hematogenous metastases
- When metastasis occurs – usually only to regional nodes and lung.
- Clinical: jaundice, increased liver size, friction rub, ascites, pain, 50-90% will have (+) alpha fetoprotein level;
- Causes of death: liver failure, GI bleeding from varices, cachexia, infection, intraperitoneal hemorrhage from rupture of one of the HCC nodules.
Tags: cardiac failure, chronic liver disease, cirrhosis, diabetes, dietary iron overload, focal nodular hyperplasia, hemochromatosis, hemosiderosis, Hepatocellular Adenoma, Hepatocellular Carcinoma, hepatocytes, Iron overload syndromes, iron-loading anemias, micronodular necrosis, neoplasms of liver, parenchymal cells, porphyria cutanea tarda, Secondary Iron Overload Syndromes, skin pigmentation
