Childhood non-Hodgkin lymphoma presenting as hemophagocytic syndrome.

An 8-year-old girl with a progressive systemic hemopagocytic syndrome was found to have non-Hodgkin lymphoma (NHL) after multiple nondiagnostic biopsies. Routine histochemistry and flow cytometry demonstrated this to be a peripheral T-cell process and cytogenetics identified a t(2;5)(p23;q35). An extensive evaluation for an infectious agent failed to identify a pathogen. Treatment according to a standard lymphoma protocol produced a rapid response and the girl remains in remission without evidence of hemophagocytic syndrome 18 months from diagnosis. In children with systenic hemophagocytosis, a diagnosis of NHL should be aggressively pursued.