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Lymphoproliferations of Immunodeficiency

  • Ethel Cesarman
  • Amy Chadburn
Chapter

Abstract

Immunodeficient patients are at an increased risk for developing lymphoproliferative disorders (LPD), including lymphomas. The World Health Organization (WHO) classification recognizes four clinical settings associated with the development of immunodeficiency-related LPDs: (1) primary immune disorders, (2) HIV infection, (3) iatrogenic immunosuppression following solid organ or allogeneic bone marrow transplantation (posttransplant lymphoproliferative disorder [PTLD]), and (4) methotrexate therapy, usually for an autoimmune disorder.1 These lesions are highly heterogeneous, largely due to the various underlying causes of the different immunodeficiencies; however, they share several features (see Table 34-1). In most instances, the LPD are related to Epstein-Barr virus (EBV or HHV-4) infection, and thus, in situations where immunocompetence can be reestablished, these EBV-driven proliferations may regress. However, the development of secondary genetic structural alterations in oncogenes and tumor suppressor genes, not all of which have been defined, results in transformation to a neoplastic process that is no longer responsive to immune modulation.

Keywords

Primary Effusion Lymphoma BCL6 Mutation IGLL Gene Burkitt Lymphoma Terminal Repeat Region 
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

© Springer Science+Business Media, LLC 2007

Authors and Affiliations

  • Ethel Cesarman
    • 1
  • Amy Chadburn
    • 2
    • 3
    • 4
  1. 1.Department of PathologyWeill Medical College of Cornell UniversityNew YorkUSA
  2. 2.Department of Pathology and Laboratory MedicineUSA
  3. 3.Immunopathology LaboratoryNew York
  4. 4.Presbyterian Hospital/Weill Medical College of Cornell UniversityNew YorkUSA

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