Lymphomatoid granulomatosis involving central nervous system successfully treated with rituximab alone.

OBJECTIVE

To report the successful treatment of a patient with lymphomatoid granulomatosis (LYG), a rare Epstein-Barr virus-positive lymphoproliferative disorder, using rituximab (anti-CD20 monoclonal antibody). The prognosis for LYG has been reported to be poor, and no satisfactory treatment has been established. Because central nervous system (CNS) involvement of LYG has been known to show poor prognosis, the establishment of an effective treatment for CNS LYG with mild adverse effects is desired.

DESIGN

Case report.

SETTING

University hospital.

PATIENT

A 48-year-old Japanese man presenting with slowly progressive spastic paraparesis diagnosed as LYG involving the CNS and lungs.

INTERVENTIONS

The patient was treated with rituximab (375 mg/m2, once weekly for 1 month) alone. Main Outcome Measure Improvement of the lesions on imaging.

RESULTS

The neurological signs resolved and the lesions in the CNS and lungs were mostly diminished after the rituximab monotherapy without any adverse effects. The patient stayed in remission for 18 months.

CONCLUSION

Rituximab monotherapy was effective in treating the patient; hence, rituximab should be considered as the initial treatment against LYG involving the CNS.