Hematopathology of X-Linked Lymphoproliferative Syndrome

  • David T. Purtilo


Recognition of the X-linked lymphoproliferative syndrome (XLP) as a discrete entity emerged during the early 1970’s. This immunodeficiency syndrome results from inherited susceptibility to Epstein-Barr virus (EBV). It is manifested by a variety of aproliferative (aplastic anemia, red cell aplasia, agranulocytosis, acquired hypo- or agammaglobulinemia) and lymphoproliferative phenotypes (fatal or chronic infectious mononucleosis, B cell lymphomas, and pseudolymphoma). I aim to: 1) provide a historical description of the events leading to the present definition of the syndrome; 2) portray morphological manifestations of the major phenotypes of XLP; and 3) briefly consider other potential oncogenic viruses which may provoke opportunistic malignancies in individuals with acquired or inherited immunodeficiency.


Aplastic Anemia Malignant Lymphoma Infectious Mononucleosis Thymus Gland Viral Oncogenesis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Plenum Publishing Corporation 1984

Authors and Affiliations

  • David T. Purtilo
    • 1
  1. 1.Department of Pathology and Laboratory Medicine Eppley Institute for Research in Cancer and Allied DiseasesUniversity of Nebraska Medical CenterOmahaUSA

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