T-cell lymphoblastic leukemia/lymphoma in Nijmegen breakage syndrome.

@article{Pasi2004TcellLL,
  title={T-cell lymphoblastic leukemia/lymphoma in Nijmegen breakage syndrome.},
  author={Srdjan S Pasi{\'c} and Dragana Vuji{\'c} and Maurilia Fiorini and Lucia Dora Notarangelo},
  journal={Haematologica},
  year={2004},
  volume={89 8},
  pages={ECR27}
}
Nijmegen breakage syndrome (NBS) is a rare autosomal-recessive disorder characterized by microcephaly, immunodeficiency and predisposition to cancer, mainly B-cell lymphomas. Our 10-years-old female patient with NBS developed T-cell lymphoblastic leukemia/lymphoma (TLBL/ALL). The use of standard chemotherapy in our patient, except for cranial irradiation, led to complete and sustained remission of TLBL/ALL. In patients with chromosomal instability syndromes chemotherapy must be modified and… CONTINUE READING
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