title: Burkitt's lymphoma in a patient with adenosine deaminase deficiency-severe combined immunodeficiency treated with polyethylene glycol-adenosine deaminase.
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.
authors.affiliation: Division of Immunology/Allergy, The Hospital for Sick Children and The University of Toronto, Toronto, Ontario, Canada.
authors.forename: Maitham
authors.initials: M
authors.lastname: Husain
authors.forename: Eyal
authors.initials: E
authors.lastname: Grunebaum
authors.forename: Ahmed
authors.initials: A
authors.lastname: Naqvi
authors.forename: Adelle
authors.initials: A
authors.lastname: Atkinson
authors.forename: Bo-Yee
authors.initials: BY
authors.lastname: Ngan
authors.forename: Alessandro
authors.initials: A
authors.lastname: Aiuti
authors.forename: Chaim M
authors.initials: CM
authors.lastname: Roifman
mesh_terms: D000243:Adenosine Deaminase / Q000172:deficiency* / Q000627:therapeutic use*; D000293:Adolescent; D002051:Burkitt Lymphoma / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D011092:Polyethylene Glycols / Q000627:therapeutic use*; D018570:Risk Assessment; D016511:Severe Combined Immunodeficiency / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D011092:Polyethylene Glycols; D000243:Adenosine Deaminase