title: Two brothers in a Malaysian family with X-linked lymphoproliferative disease--a case report.
abstract: We report the occurrence of X-linked lymphoproliferative disease (XLP) in two brothers in a Malaysian family. In this disorder, a primary Epstein-Barr virus (EBV) infection is followed by an abnormal proliferation of transformed B-cells that cannot be controlled by suppressor T-cells, leading to the development of deranged immune function. This results in fatal infectious mononucleosis, acquired hypogammaglobulinaemia, virus-infected haemophagocytic syndrome and non-Hodgkin's lymphoma. The diagnosis should be considered when there is a family history of any male having a fulminant course of infectious mononucleosis, an otherwise benign disease. Early diagnosis is important as bone marrow transplantation is the only curative option in this disorder.
authors.affiliation: Department of Paediatrics, University Hospital, Kuala Lumpur, Malaysia.
authors.forename: A
authors.initials: A
authors.lastname: Hany
authors.forename: M K
authors.initials: MK
authors.lastname: Thong
authors.forename: H P
authors.initials: HP
authors.lastname: Lin
mesh_terms: D002675:Child, Preschool; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D006801:Humans; D007244:Infectious Mononucleosis / Q000175:diagnosis* / Q000503:physiopathology; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000235:genetics*; D008297:Male