Background: Nijmegen Breakage Syndrome (NBS) is a rare autosomal recessive DNA repair disorder characterized\nby immune deficiency, microcephaly, mental retardation and a disposition for the development of hematological\nmalignancies. So far, mostly pediatric patients have been described, since the underlying condition is often fatal\nbefore adulthood. Many patients diagnosed with Hodgkin lymphoma (HL) due to this DNA repair defect receive\nreduced treatment followed by early progression and fatal outcome.\nCase presentation: We describe here a 26-year old male caucasian patient with NBS who presented with multi\norgan failure due to HL. Immediate intensive chemotherapy lead to complete remission and reversed organ failure.\nConclusion: We show that application of standard chemotherapy can lead to long-term disease free survival in\npatients with a DNA repair disorder. Furthermore, we describe here, to the best of our knowledge, the first adult\npatient with NBS and HL.
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