X-linked lymphoproliferative disorder (XLP)
also see B-cell defects, EBV Lymphoproliferative d/o

• AKA “Duncan’s disease”
• Problem: Inadequate immune response to infection with EBV
• Get uncontrolled lymphoproliferation with EBV infection
• Genetics: X-linked, Xq25

Presentation:

• Healthy male until they acquire EBV infection
• Mean age < 5yo
• 75% develop severe EBV infection
• 80% mortality (due to extensive liver necrosis)

Prognosis

• If survive the primary infection, most develop
  • global cellular immune defects involving T, B, and NK cells
  • Lymphomas
  • Hypogammaglobulinemia

CHLA Board Review 2005