X-linked lymphoproliferative disease (Duncan disease)

X-linked lymphoproliferative disorder is initiated after an infection with the Epstein-Barr virus (EBV). EBV is the causative agent of infectious mononucloeosis, and may produce one or more of the following: signs of activation in lymphocytes and histiocytes and/or progressive hypogammaglobulinemia and/or lymphomas.

Alternative names

Lyp

Lymphoproliferative disease; XLPD; XLP

Immunodeficiency, X-linked progressive combined variable

Duncan disease

Familial fatal Epstein-barr virus infection

EBV susceptibility; EBVS

Susceptibility to infectious mononucleosis

Immunodeficiency 5; IMD5

Purtilo syndrome

X-linked lymphoproliferative syndrome (SH2D1A/SLAM-associated protein (SAP)

Classification

  • Other well-defined immunodeficiency syndromes

Inheritance

X-linked

OMIM

#308240 Lymphoproliferative syndrome

*300490 SH2 domain protein 1A; SH2D1A

Cross references

Incidence

Incidence is not known.