Neonatal Hemochromatosis

 Liver

image Pancreas
image Heart
image Thyroid
image Minor salivary glands
• Abnormal iron studies
• Liver and multiorgan failure

Macroscopic

• Shrunken liver
• Cirrhosis

Microscopic

• Marked lobular necrosis with collapse
• Regenerative nodules
• Pseudoacinar formation
• Intracanalicular bile plugs
• Giant hepatocytes
• Iron deposition in hepatocytes and ductules as well as in organs outside of liver

Top Differential Diagnoses

• Virus infection
image CMV
image Echovirus
image Herpes simplex virus
• Neonatal lupus
• Tyrosinemia
image
Lobular NecrosisH&E in this case of neonatal hemochromatosis shows lobular necrosis and collapse of the hepatocellular cords with residual hepatocytes and bile ductules.

image
Perl Iron StainPerl iron stain in this case of neonatal hemochromatosis shows marked iron deposition image within the hepatocytes.
image
Submassive Hepatocellular NecrosisHigher power of this case of neonatal hemochromatosis shows submassive hepatocellular necrosis image with a rim of residual hepatocytes image .
image
Giant CellsGiant cells are seen in this case of neonatal hemochromatosis.

TERMINOLOGY

Abbreviations

• Neonatal hemochromatosis (NH)

Synonyms

• Congenital hemochromatosis
• Neonatal iron storage disease
• Gestational alloimmune disease

Definitions

• Severe liver disease with iron overload in liver and other organs (distribution similar to hereditary hemochromatosis)
image Fetal or perinatal onset

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  4. Biliary Atresia

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Apr 20, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Neonatal Hemochromatosis

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