ABSTRACT
The primary immune disorders are rare and have diverse underlying pathologies. They occur predominantly in childhood, although common variable immunodeficiency (CVID) is seen in adults.
Fatal infectious mononucleosis is seen in patients with X-linked lymphoproliferative disorder (XLP or Duncan syndrome) and with severe combined immunodeficiency (SCID). Infection with EBV is associated with unrestrained proliferation of EBV-positive plasmacytoid and immunoblastic cells at nodal and extranodal sites. Death may be
caused by haemophagocytic syndrome, pancytopenia and infection.
