T-cell ALL in Ataxia Telangiectasia Cured With Only 7 Weeks of Anti-leukemic Therapy

Journal of Pediatric Hematology/Oncology, Wolters Kluwer, ISSN 1077-4114

Volume 37, 2, 2015

DOI:10.1097/mph.0000000000000151, Dimensions: pub.1003630265, PMID: 24663073,



  1. (1) Rigshospitalet, grid.475435.4, Capital Region






A 20-month-old girl diagnosed with T-cell acute lymphoblastic leukemia was treated according to the Nordic NOPHO ALL2000 protocol. The patient developed severe immunosuppression and experienced life-threatening adenovirus infection, which was treated with ribavirin and cidofovir. α-fetoprotein was 20- to 30-fold elevated, and genetic analysis confirmed the diagnosis of ataxia telangiectasia. Despite receiving only 7 weeks of anti-leukemic therapy, she has stayed in first remission now 8 years after the diagnosis. We speculate that this could be because of increased chemosensitivity of ATM-mutated leukemic cells, adenovirus causing a direct oncolytic effect, and/or high levels of endogenous cortisol during her severe infection.

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Times Cited: 5

Field Citation Ratio (FCR): 1.1

Relative Citation ratio (RCR): 0.13