Ataxia-telangiectasia (A-T) is an autosomal recessive disorder characterized by cerebellar ataxia, telangiectasia, immune defect, and predisposition to leukemia/lymphoma. Because of their hypersensitivity to DNA-damaging agents, patients with A-T may require special consideration. However, an optimal strategy for these patients has not been established. Here, we describe an A-T female with diffuse large B-cell lymphoma successfully treated with rituximab combined with reduced-dose chemotherapy. Given the high incidence of hematopoietic malignancies in patients with A-T, and the hypersensitivity of these patients to DNA-damaging agents, we discuss whether a reduced-dose chemotherapy with a molecular targeting agent may be of merit.
*Department of Pediatrics and Developmental Biology, Graduate School of Medicine, Tokyo Medical and Dental University
†Department of Pediatrics and Adolescent Medicine, School of Medicine, Juntendo University, Tokyo, Japan
The authors declare no conflict of interest.
Reprints: Masatoshi Takagi, MD, PhD, Department of Pediatrics and Developmental Biology, Tokyo Medical and Dental University, 1-5-45 Bunkyo-ku, Yushima, Tokyo 113-8519, Japan (e-mail: firstname.lastname@example.org).
Received January 13, 2012
Accepted June 29, 2012