In Nijmegen breakage syndrome (NBS), malignant disease can arise at an early age. When disease is refractory to initial treatment or relapses, a structured protocol defines the next therapeutic step — with specific approaches for paediatric patients and for those with EBV-driven lymphoid malignancies.
This protocol applies to patients with NBS presenting with refractory or relapsed malignant disease, including children aged one year or older and the subset with EBV-associated lymphoid malignancies requiring salvage intervention.
The protocol involves molecular targeted therapy, with a defined agent for eligible paediatric patients and a separate salvage strategy for the EBV-driven lymphoid malignancy subset. The complete regimen, eligibility criteria, sequencing, and decision algorithm are available in the full protocol.