Epstein Barr virus induced lymphoma in a child with IPEX syndrome

Pediatr Blood Cancer. 2008 May;50(5):1056-7. doi: 10.1002/pbc.21341.

Abstract

IPEX syndrome (immune deficiency, polyendocrinopathy, enteropathy, X-linked) is a disorder or regulatory T cell (Treg) function which can result in early death due to infection or complications related to autoimmunity. Therapeutic options for these patients can include allogeneic stem cell transplantation (SCT) or the use of immunosuppressive regimens to control the manifestations of autoimmunity. We report a patient with IPEX syndrome who was managed with rapamycin and subsequently developed EBV induced lymphoma.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Epstein-Barr Virus Infections / drug therapy
  • Epstein-Barr Virus Infections / virology*
  • Genetic Diseases, X-Linked / complications*
  • Genetic Diseases, X-Linked / pathology
  • Genetic Diseases, X-Linked / therapy
  • Herpesvirus 4, Human / pathogenicity*
  • Humans
  • Immunologic Deficiency Syndromes / complications*
  • Immunologic Deficiency Syndromes / pathology
  • Immunologic Deficiency Syndromes / therapy
  • Immunosuppressive Agents / therapeutic use
  • Infant
  • Lymphoma / drug therapy
  • Lymphoma / virology*
  • Male
  • Polyendocrinopathies, Autoimmune / complications*
  • Polyendocrinopathies, Autoimmune / pathology
  • Polyendocrinopathies, Autoimmune / therapy
  • Protein-Losing Enteropathies / complications*
  • Protein-Losing Enteropathies / pathology
  • Protein-Losing Enteropathies / therapy
  • Sirolimus / therapeutic use
  • Syndrome

Substances

  • Immunosuppressive Agents
  • Sirolimus