Which histologic findings are characteristics of X-linked lymphoproliferative (XLP) syndrome?

Updated: Jan 27, 2019
  • Author: Karen Seiter, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Liver biopsy results typically show an intense periportal B-cell lymphoid infiltrate containing EBV-nuclear antigen (EBNA-1) often surrounded by numerous CD8-positive T lymphocytes and natural killer cells. In later stages, periportal necrosis is observed in most patients. Other organs that can be involved include the liver, heart, brain, and thymus. Findings in the bone marrow are generally reactive.


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