Category: Image Based Question

A. Ulcerative Colitis

B. Psoriasis

C. Diabetes Mellitus

D. Hypercholesterolemia

Correct Answer » C Explanation

Ans:C. Diabetes Mellitus

Image shows: Hereditary hemochromatosis. In this Prussian blue– stained histologic section, hepatocellular iron appears blue. The parenchymal architecture is normal.

Hemochromatosis

• Hereditary hemochromatosis refers to genetic disorders characterized by excessive accumulation of body iron, most of which is deposited in the liver, pancreas, and heart.

• The hereditary hemochromatosis gene, responsible for the most common form of this disorder, is called HFE. It is located on the short arm of chromosome 6.

• Acquired forms of iron accumulation from known sources of excess iron are called secondary iron overload. Among the most important are multiple transfusions, ineffective erythropoiesis (as in β-thalassemia and myelodysplastic syndromes), and increased iron intake.

• Fully developed cases show (1) cirrhosis (seen in all patients), (2) diabetes mellitus (in 75% to 80% of patients), and (3) skin pigmentation (in 75% to 80%).

Morphology:

• The morphologic changes in hereditary hemochromatosis are all responses to the deposition of hemosiderin in the following organs (in decreasing order of severity): liver, pancreas, myocardium, pituitary, adrenal, thyroid and parathyroid glands, joints, and skin.

• In the liver, iron becomes evident first as golden-yellow hemosiderin granules in the cytoplasm of periportal hepatocytes, which stain blue with the Prussian blue stain.

• With increasing iron load, there is progressive involvement of the rest of the lobule, along with bile duct epithelium and Kupffer cells.

• Iron is a direct hepatotoxin, and inflammation is characteristically absent. At this stage, the liver typically is slightly larger than normal, dense, and chocolate brown.

• Fibrous septa develop slowly, linking portal tracts to each other and leading ultimately to cirrhosis in an intensely pigmented (very dark brown to black) liver

A. Autosomal Recessive

B. Autosomal Dominant

C. X linked recessive

D. X linked dominant

Correct Answer » A Explanation

Ans:A. Autosomal recessive.

Image shows: α1-Antitrypsin deficiency. Periodic acid–Schiff stained histologic section of liver, highlighting the characteristic magenta cytoplasmic granules.

α1-Antitrypsin Deficiency.

• α1-Antitrypsin (AAT) deficiency is an autosomal recessive disorder marked by abnormally low serum levels of this protease inhibitor.

• The major function of AAT is the inhibition of proteases, particularly neutrophil elastase released at sites of inflammation.

• AAT deficiency leads to pulmonary emphysema, because a relative lack of this protein permits the unrestrained activity of tissue-destructive proteases.

• Hepatic disease results from retention of mutant AAT in the liver.

MORPHOLOGY

• Hepatocytes in AAT deficiency contain round to oval cytoplasmic globules composed of retained AAT, a glycoprotein that is strongly positive in a periodic acid–Schiff stain .

• By electron microscopy they lie within smooth, and sometimes rough, endoplasmic reticulum

A. Alcohol

B. Alpha 1 Antitrypsin deficiency

C. Hepatitis C virus

D. Constrictive pericarditis

Correct Answer » D Explanation

Ans:D. Constrictive pericarditis

Image shows: Centrilobular hemorrhagic necrosis (nutmeg liver). The cut liver section, in which major blood vessels are visible, is notable for a variegated mottled red appearance, representing hemorrhage in the centrilobular regions of the parenchyma.

NUTMEG LIVER

• A nutmeg liver appearance is due to a perfusion abnormality of the liver usually as result of hepatic venous congestion.

Conditions associated with hepatic venous congestion include:

1. hepatic veno-occlusive disease
2. Budd-Chiari syndrome
3. congestive hepatopathy
4. right heart failure
5. constrictive pericarditis

A. Budd Chiari Syndrome

B. Congestive hepatopathy

C. Hepatocellular Carcinoma

D. Alpha 1 Antitrypsin deficiency

Correct Answer » A Explanation

Ans:A. Budd Chiari Syndrome.

Image shows: Budd-Chiari syndrome. Thrombosis of the major hepatic veins has caused profound hepatic congestion.

Hepatic Vein Thrombosis (Budd-Chiari Syndrome) .

• The Budd-Chiari syndrome results from the thrombosis one or more major hepatic veins and is characterized by hepatomegaly, ascites, and abdominal pain.

• Hepatic vein thrombosis is associated with myeloproliferative disorders (especially polycythemia vera), pregnancy, the postpartum state, the use of oral contraceptives, paroxysmal nocturnal hemoglobinuria, and intra-abdominal cancers, particularly hepatocellular carcinoma.

MORPHOLOGY

• With acutely developing thrombosis of the major hepatic veins or inferior vena cava, the liver is swollen and red-purple, with a tense capsule .

• On microscopic examination, severe centrilobular congestion and necrosis are apparent in affected hepatic parenchyma. In instances in which the thrombosis develops more slowly, centrilobular fibrosis is seen.

• The major veins may contain completely or incompletely occlusive fresh thrombi or, in chronic cases, organized adherent thrombi.

Identify this testicular tumor: