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

This liver biopsy shows sinusoidal dilatation with prominent Kupffer cells


Hypertrophic Kupffer cells




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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