title: Successful treatment of hodgkin lymphoma in nijmegen breakage syndrome.
abstract: Nijmegen breakage syndrome (NBS) is a rare DNA repair disorder characterized by microcephaly, immunodeficiency, and predisposition to malignancy. We report on a 5-year-old patient with NBS who presented with nodular sclerosing type of Hodgkin disease stage IVB. Chemotherapy consisting of COPP/ABV regimen with reduction at 75% of full doses was employed. During this treatment, no major toxic or infectious complications were observed. Complete remission was achieved lasting now for 20 months. In DNA repair disorders, prognosis of Hodgkin disease is poor as opposed to excellent overall prognosis in general pediatric population. Better survival may be achieved both with adopted, disease-specific regimens, and individualized approach considering patient's clinical condition. Also, better recognition and treatment of infections during chemotherapy may reduce early deaths in patients with DNA repair disorders.
authors.affiliation: Departments of Pediatric Hematology, Pulmonology, Medical Genetics, Pathology, and Immunology, Mother and Child Health Institute, Belgrade, Serbia.
authors.forename: Ankica
authors.initials: A
authors.lastname: Jovanovic
authors.forename: Predrag
authors.initials: P
authors.lastname: Minic
authors.forename: Marija
authors.initials: M
authors.lastname: Scekic-Guc
authors.forename: Slavisa
authors.initials: S
authors.lastname: Djuricic
authors.forename: Sanja
authors.initials: S
authors.lastname: Cirkovic
authors.forename: Corry
authors.initials: C
authors.lastname: Weemaes
authors.forename: Srdjan
authors.initials: S
authors.lastname: Pasic
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001761:Bleomycin / Q000627:therapeutic use; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D006689:Hodgkin Disease / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D049932:Nijmegen Breakage Syndrome / Q000150:complications / Q000188:drug therapy*; D011241:Prednisone / Q000627:therapeutic use; D011344:Procarbazine / Q000627:therapeutic use; D012074:Remission Induction; D014747:Vinblastine / Q000627:therapeutic use; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D001761:Bleomycin; D011344:Procarbazine; D014750:Vincristine; D014747:Vinblastine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone