Hematopathology and pathogenesis of the X-linked recessive lymphoproliferative syndrome

Demonstration of Epstein-Barr virus DNA in a previously healthy boy with fulminant hepatic failure

“…Massive hepatic necroses have rarely been seen in adults [1,2] and in children without immunodeficiencies [10,15]. Histological examination of the liver in our patient suggested EBV as a cause of the liver disease, there were no indications of drug-induced liver disease [7,14,21,27].…”

“…In male patients with X-linked lymphoproliferative syndrome a high incidence of massive hepatic necrosis during EBV infections was encountered [15,16]. Purtilo suggested that immunodeficiency states are necessary for development of an acute liver failure [16].…”

“…This causes a T-cell and antibody response to terminate the infection [26]. Some T-cell defects apparently induced by EBV [4,13] and the frequency of massive hepatic necrosis in patients with X-linked lymphoproliferative syndrome and EBV infection [15,16] support this theory.…”

“…It seems to occur more frequently in patients with X-linked recessive lymphoproliferative syndrome [15,16]. In the past, demonstration of a causal relation between EBV and liver cell necrosis was not possible.…”

Fatal lymphoproliferation and acute monocytic leukemia-like disease following infectious mononucleosis in the elderly

“…In immunodeficient persons infectious mononucleosis may run a fulminating, frequently fatal course with non-proliferative (aplastic anemia, agranulocytosis, hypogammaglobulinemia) and proliferative (American Burkitt's lymphoma, immunoblastic sarcoma of B-ceils, fatal infectious mononucleosis, plasmocytoma) phenotypes (Purtilo et al 1979). This immunodeficiency may occur sporadic or clustered in certain families.…”