Epstein-Barr virus-associated lymphoproliferative syndromes: studies in two European families.
The X-linked lymphoproliferative syndrome (XLP), also known as Duncan's disease, is an X-linked recessive disorder that is characterized by the inability of affected individuals to mount a sufficient immune response to Epstein-Barr virus (EBV). After EBV primary infection, male family members suffer from severe infectious mononucleosis (IM), aplastic anemia, hypogammaglobulinemia, and a spectrum of lymphoproliferative diseases. Autosomal mode of inheritance with similar symptoms as in XLP has been reported. We have studied two families with EBV-associated syndromes and an X-linked and an autosomal mode of inheritance, respectively. Affected family members presented with severe IM, hemophagocytosis, aplastic anemia, acquired hypogammaglobulinemia, and B-cell lymphoproliferative diseases.
Schuster
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V
Department of Pediatrics, University of Würzburg, FRG.
Dohrmann
E
E
Kreth
H W
HW
D000361:Agammaglobulinemia / Q000209:etiology; D000741:Anemia, Aplastic / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D008040:Genetic Linkage; D004854:Herpesvirus 4, Human; D006801:Humans; D007223:Infant; D007244:Infectious Mononucleosis / Q000235:genetics* / Q000382:microbiology; D008232:Lymphoproliferative Disorders / Q000235:genetics* / Q000382:microbiology; D008297:Male; D010375:Pedigree