Catalog

X-linked lymphoproliferative disease

No MMS code

Link to WHO Definition

X-linked lymphoproliferative disease – http://id.who.int/icd/entity/2126467634

X-linked lymphoproliferative disease is a hereditary immunodeficiency characterised, in the majority of cases, by an inadequate immune response to infection with the Epstein-Barr virus (EBV). Infection with the Epstein-Barr virus can result in one or several of the following manifestations: fulminant infectious mononucleosis, macrophage-activation syndrome or hemophagocytic lymphohistiocytosis (HLH; see this term), and/or progressive hypogammaglobulinemia and/or lymphomas.