title: Successful treatment of diffuse large B-cell non-hodgkin lymphoma with modified CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) chemotherapy and rituximab in a patient with Nijmegen syndrome.
abstract: A 17-year-old Croatian boy with Nijmegen breakage syndrome (NBS) who developed diffuse large B-cell non-Hodgkin lymphoma is presented. The majority of the patients with this rare autosomal recessive disease are of Slavic origin and, in most of them, the disease is caused by NBS1 mutation 657del5, as was found in our patient. Nijmegen breakage syndrome is characterized by microcephaly, growth retardation, abnormal facial appearance, spontaneous chromosomal rearrangements, immunodeficiency, and a high predisposition to cancer development, predominantly lymphoma. Because of increased sensitivity to radiation therapy and chemotherapy, the treatment of malignancies in patients with NBS can be difficult. To our knowledge, our patient is the first with NBS reported in the literature who was successfully treated for diffuse large B-cell lymphoma with the anti-CD20 monoclonal antibody rituximab in addition to a modified dose of CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) chemotherapy. He has been in complete remission for 3 years after finishing the treatment.
authors.affiliation: Department of Pediatrics, University Hospital Center Zagreb, Croatia. drdumic@mef.hr
authors.forename: Miroslav
authors.initials: M
authors.lastname: Dumic
authors.forename: Ivo
authors.initials: I
authors.lastname: Radman
authors.forename: Nevena
authors.initials: N
authors.lastname: Krnic
authors.forename: Marin
authors.initials: M
authors.lastname: Nola
authors.forename: Rajko
authors.initials: R
authors.lastname: Kusec
authors.forename: Davor
authors.initials: D
authors.lastname: Begovic
authors.forename: Boris
authors.initials: B
authors.lastname: Labar
authors.forename: Marko
authors.initials: M
authors.lastname: Rados
mesh_terms: D000293:Adolescent; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage / Q000031:analogs & derivatives; D006801:Humans; D007621:Karyotyping; D016393:Lymphoma, B-Cell / Q000150:complications* / Q000188:drug therapy*; D008297:Male; D049932:Nijmegen Breakage Syndrome / Q000150:complications* / Q000235:genetics; D011241:Prednisone / Q000008:administration & dosage; D000069283:Rituximab; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone