Autosomal recessive inheritance
• Hyperproduction of cytokines and chemokines due to T-cell dysregulation that causes “cytokine storm” with proliferation and activation of macrophages
Ancillary Tests
![image](/wp-content/uploads/2017/04/image02569-3.jpeg)
This liver biopsy shows sinusoidal dilatation with prominent Kupffer cells
![image](/wp-content/uploads/2017/04/image02570-16.jpeg)
![image](/wp-content/uploads/2017/04/image02571-4.jpeg)
Hypertrophic Kupffer cells
![image](/wp-content/uploads/2017/04/image02572-1.jpeg)
![image](/wp-content/uploads/2017/04/image02573.jpeg)
![image](/wp-content/uploads/2017/04/image02574-1.jpeg)
![image](/wp-content/uploads/2017/04/image02575-2.jpeg)
Immunohistochemical stains using histiocytic markers, such as CD163, can highlight hyperplastic and hypertrophic Kupffer cells, but the stains do not help in the recognition of hemophagocytosis.
ETIOLOGY/PATHOGENESIS
Primary or Familial HLH
• Autosomal recessive inheritance
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