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Burkitt's lymphoma in a patient with adenosine deaminase deficiency-severe combined immunodeficiency treated with polyethylene glycol-adenosine deaminase.
Husain, Maitham; Grunebaum, Eyal; Naqvi, Ahmed; Atkinson, Adelle; Ngan, Bo-Yee; Aiuti, Alessandro; Roifman, Chaim M.
J Pediatr ; 151(1): 93-5, 2007 Jul.
Article in English | MEDLINE | ID: mdl-17586199

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.


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