Burkitt's lymphoma in a patient with adenosine deaminase deficiency-severe combined immunodeficiency treated with polyethylene glycol-adenosine deaminase

J Pediatr. 2007 Jul;151(1):93-5. doi: 10.1016/j.jpeds.2007.03.059.


We describe a patient with severe combined immunodeficiency because of aberrations in adenosine deaminase (ADA) who despite adequate replacement with polyethylene glycol-linked ADA (PEG-ADA) for 13 years developed Burkitt's lymphoma. Although treatment corrected the metabolic abnormalities caused by ADA deficiency, it failed to fully restore cellular immunity.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adenosine Deaminase / deficiency*
  • Adenosine Deaminase / therapeutic use*
  • Adolescent
  • Burkitt Lymphoma / complications*
  • Burkitt Lymphoma / diagnosis
  • Burkitt Lymphoma / drug therapy
  • Dose-Response Relationship, Drug
  • Drug Administration Schedule
  • Female
  • Follow-Up Studies
  • Humans
  • Polyethylene Glycols / therapeutic use*
  • Risk Assessment
  • Severe Combined Immunodeficiency / complications*
  • Severe Combined Immunodeficiency / diagnosis
  • Severe Combined Immunodeficiency / drug therapy*
  • Severity of Illness Index
  • Treatment Outcome


  • Polyethylene Glycols
  • Adenosine Deaminase