Abstract

Lymphocytic cholangitisis a rare Porto-hepatic disease with predominant infiltration of lymphocytes and plasma cells in the portal tracts of the liver. X-linked lymphoproliferative disease is an inborn error of immunity caused by a defect in the signaling lymphocyte-activation molecule (SLAM)-associated protein. Lymphocytic cholangitis is reported in feline species, however, reports in humans is scarce. We report the first case of Epstein-Barr Virus-related lymphocytic cholangitis in a child with X-linked lymphoproliferative syndrome.

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