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

Maitham Husain; Eyal Grunebaum; Ahmed Naqvi; Adelle Atkinson; Bo-Yee Ngan; Alessandro Aiuti; Chaim M Roifman

doi:10.1016/j.jpeds.2007.03.059

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.

Authors

Maitham Husain¹, Eyal Grunebaum, Ahmed Naqvi, Adelle Atkinson, Bo-Yee Ngan, Alessandro Aiuti, Chaim M Roifman

Affiliation

¹ Division of Immunology/Allergy, The Hospital for Sick Children and The University of Toronto, Toronto, Ontario, Canada.

PMID: 17586199
DOI: 10.1016/j.jpeds.2007.03.059

Abstract

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

Substances

Polyethylene Glycols
Adenosine Deaminase

Grants and funding

TGT06S01/TI_/Telethon/Italy