Successful treatment of hodgkin lymphoma in nijmegen breakage syndrome

Journal of Pediatric Hematology/oncology
Ankica JovanovicSrdjan Pasic

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.

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Citations

Aug 18, 2009·Bone Marrow Transplantation·M H AlbertB H Belohradsky
Mar 1, 2012·Orphanet Journal of Rare Diseases·Krystyna H ChrzanowskaMartin Digweed
Jan 17, 2014·BMC Hematology·Katharina EngelUlrich Keller
Dec 21, 2010·The Journal of Allergy and Clinical Immunology·Saul Oswaldo Lugo ReyesJacinta Bustamante

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